Materiały źródłowe

• #1 Updates on the diagnosis and management of multicentric Castleman disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7821823/

  Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disease. The diagnosis of this disease is typically challenging and requires collaboration between clinicians and pathologists. Moreover, it is important to exclude other diseases (such as malignancies, autoimmune diseases, and infectious diseases) that have similar clinical manifestations and pathological findings. […] The diagnosis of MCD is based on the clinical signs of systemic inflammation, serological tests, and typical pathological features. […] Given the diverse symptoms and laboratory data, lymph node biopsy is indispensable for establishing the diagnosis of MCD. The biopsies of the lymph nodes are evaluated for the histologic features, immunostaining, and special staining for pathogens. […] A definitive diagnosis of MCD should be established after excluding the above diseases.

• #1 Diagnosis – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/diagnosis-treatment/

  Castleman disease (CD) can be difficult to diagnose as it is both a rare disease and an imitator of many other diseases. Patients can have the same symptoms as some cancers and some autoimmune diseases. Due to the complexity of the disease, physicians often need to rule out many other diseases before CD is suspected. […] Therefore, patients must undergo an excisional lymph node biopsy to be diagnosed with CD. This is absolutely necessary so that patients with other diseases are not incorrectly diagnosed with Castleman disease. […] According to our international, evidence-based guidelines for UCD and iMCD, the tests needed for diagnosis should include: […] Lymph node biopsy: A doctor takes a sample of tissue from a lymph node and looks at it under a microscope to identify features of Castleman disease. […] Castleman Disease can only be diagnosed through biopsy (removing an enlarged lymph node) and examination under the microscope to identify features of Castleman disease.

• #1 Castleman Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK576394/

  Castleman disease is a lymphoproliferative disorder with a nondefinite etiology and can be classified into subtypes based on clinical and histological manifestations. […] The diagnosis of Castleman disease is always a challenge in clinical experience, as it does not have specific features that could be distinguished from other diseases causing lymphadenopathies. Therefore, the diagnosis should be finalized when the patient meets both major criteria, at least two of the minor criteria and one laboratory abnormality. […] Histopathologic screening of lymph nodes is done to assess a single node involvement (suggestive of UCD) or a multimode involvement (suggesting MCD) after excluding other infectious, malignant, and autoimmune disorders that exhibit their characteristic features in the lymph nodes.

• #1 Castleman disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/castleman-disease/diagnosis-treatment/drc-20543055

  After reviewing your medical history and doing a detailed physical exam, your healthcare professional may recommend: […] Blood and urine tests. These help rule out other infections or diseases. These tests also can find anemia and changes in blood proteins that can be typical of Castleman disease. […] Imaging tests. These tests can find enlarged lymph nodes or an enlarged liver or spleen. A CT scan of the neck, chest, abdomen and pelvis may be used. A positron emission tomography scan, also known as a PET scan, may be used to diagnose Castleman disease. PET scans also can show whether a treatment is working. […] Lymph node biopsy. This test is essential to diagnose Castleman disease and rule out related disorders, such as lymphoma. In a biopsy, a tissue sample from an enlarged lymph node is removed and looked at in a laboratory.

• #1 Castleman Disease | Condition | UAMS Health

  https://uamshealth.com/condition/castleman-disease/

  Castleman Disease can only be diagnosed through biopsy (removing an enlarged lymph node) and examination under the microscope to identify features of Castleman disease. […] According to international, evidence-based guidelines for UCD and iMCD from the Castleman Disease Collaborative Network, these tests should include: Lymph Node Biopsy: A doctor takes a sample of tissue from a lymph node and looks at it under a microscope to identify features of Castleman disease. […] Laboratory tests: Complete blood count, inflammation markers (CRP/ESR), and markers of organ function such as liver function tests, albumin, creatinine as well as HIV/HHV8 testing for HHV-8-associated MCD. […] Imaging tests: Tests such as X-rays and PET-CT scans allow doctors to locate enlarged lymph nodes and activity of those lymph nodes in the body.

• #1 Diagnosis and Management of Castleman Disease- Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/features/diagnosis-and-management-of-castleman-disease/2/

  Castleman disease is a pathological diagnosis made by excisional biopsy of affected lymph node tissue. In cases of deeper or less accessible disease, core needle biopsy is preferred to fine needle aspiration, because fine needle aspirations are insensitive for both UCD and MCD. […] From a pathological standpoint, Castleman disease is a diagnosis of exclusion and its varied histological features give rise to a broad differential diagnosis that includes both benign and neoplastic entities, most of which can be excluded on the basis of careful histological examination, immunohistochemical, or other ancillary studies (eg, flow cytometry, molecular genetics) and correlation with clinical, laboratory, and radiological findings. […] In all of these scenarios, the diagnosis of Castleman disease can be made based on the lack of additional histological features supporting a diagnosis of lymphoma and the absence of a clonal B-cell population with a characteristic immunophenotype.

• #1 Idiopathic multicentric Castleman disease – Wikipedia

  https://en.wikipedia.org/wiki/Idiopathic_multicentric_Castleman_disease

  Diagnosis requires exclusion of diseases that can present with similar clinical findings and similar appearance on microscopic analysis of tissue from an enlarged lymph node. Diseases that must be excluded in the diagnosis of iMCD include infectious diseases, such as HHV-8-associated MCD, Epstein-Barr virus mononucleosis, and reactive lymphadenopathy; autoimmune diseases, such as systemic lupus erythematosus and rheumatoid arthritis; and cancers, including lymphoma, multiple myeloma, and primary lymph node plasmacytoma.

• #1 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Elevation of serum IgG4 levels or increased IgG4 positive cells in the lymph nodes can be observed, not only in IgG4-related disease, but also in Castleman disease. […] Human herpesvirus type 8 (human herpesvirus-8, HHV-8; Kaposi’s sarcoma-associated herpesvirus, KSHV) associated multicentric Castleman disease can be diagnosed by the typical histopathological features and the presence of the HHV-8 genome in the blood or in the lymph nodes. […] With respect to iMCD, the following international diagnostic criteria have been proposed. […] iMCD is diagnosed when satisfying two major criteria and two or more items including at least one of the test criteria out of the 11 small criteria as well as diseases defined by exclusion criteria are excluded. […] Major Criteria (need both): Histopathologic lymph node features consistent with the iMCD spectrum.

• #1 Unicentric Castleman disease – UpToDate

  https://www.uptodate.com/contents/unicentric-castleman-disease

  Castleman disease (CD, angiofollicular lymph node hyperplasia) describes a heterogeneous group of lymphoproliferative disorders that share common histopathologic features. […] CD is classified into at least three distinct disorders that have varying clinical features, treatments, and outcomes. Classification is based on the number of regions of enlarged lymph nodes with characteristic histopathologic features and the presence/absence of human herpesvirus 8 (HHV-8, also called Kaposi sarcoma-associated herpesvirus [KSHV]) infection. […] Unicentric CD (UCD) involves one or more enlarged lymph node(s) in a single region of the body that demonstrates CD histopathologic features. […] Emerging evidence suggests that some patients with CD do not meet the criteria for UCD or MCD. The term „oligocentric CD” has been proposed for patients who have enlarged lymph nodes in two to three adjacent lymph node stations but do not meet the laboratory and clinical minor criteria required to diagnose idiopathic MCD. More research is needed, but clinical manifestations and treatment approaches for oligocentric CD appear to be more similar to UCD than MCD.

• #1 Castleman disease | City of Hope

  https://www.cancercenter.com/risk-factors/castleman-disease

  Most UCD patients dont experience whole-body symptoms, and many times the enlarged lymph nodes are discovered by accident, during a doctor visit for another condition. A diagnosis typically involves imaging tests such as a computed tomography (CT) scan, ultrasound or magnetic resonance imaging (MRI), as well as a biopsy, which is sent to a lab for evaluation. […] While theres no set diagnostic criteria for MCD, its usually diagnosed if a patient has: […] A biopsy is essential to make the right diagnosis because the lymph node features found in Castleman disease may also be seen in other diseases, including cancers like lymphoma and autoimmune diseases such as lupus and rheumatoid arthritis.

• #1 HHV-8-negative/idiopathic multicentric Castleman disease – UpToDate

  https://www.uptodate.com/contents/hhv-8-negative-idiopathic-multicentric-castleman-disease

  Castleman disease (CD, angiofollicular lymph node hyperplasia) describes a heterogeneous group of lymphoproliferative disorders that share common histopathologic features. […] CD is classified into at least three distinct disorders that have varying clinical features, treatments, and outcomes. Classification is based on the number of regions of enlarged lymph nodes with characteristic histopathologic features and the presence/absence of human herpesvirus 8 (HHV-8, also called Kaposi sarcoma-associated herpesvirus [KSHV]) infection. […] Multicentric CD (MCD) involves multiple regions of lymphadenopathy that demonstrate histopathologic features consistent with CD. These patients also have systemic inflammatory symptoms with generalized lymphadenopathy, hepatosplenomegaly, cytopenias, and organ dysfunction due to excessive proinflammatory hypercytokinemia, often including interleukin (IL)-6. MCD is further subclassified according to the presence of HHV-8 into HHV-8-associated MCD and idiopathic MCD (HHV-8-negative MCD).

• #1 Castleman’s Disease Presents Challenges in Diagnosis, Treatment | Duke Health Referring Physicians

  https://physicians.dukehealth.org/articles/castlemans-disease-presents-challenges-diagnosis-treatment

  Castlemans diseasea rare, complex condition existing at the diagnostic intersection of hematology, oncology, rheumatology, and infectious diseaseis now better understood but remains difficult to identify and treat early in its course. […] Laboratory findings for Castlemans disease are often confusing, says Duke rheumatologist Philip Chu, MD, a second-year fellow who has studied the disease pathogenesis. […] Although Castlemans disease can be strongly suggested by biopsy of an enlarged lymph node, like many rheumatological conditions, it cannot be identified with a single lab result. […] Specialists reach the diagnosis of Castlemans disease after a thorough review of clinical presentation, laboratory data, and differential diagnoses, Chu says. […] The lab and imaging data may initially suggest one diagnosis, but if the patient is not responding to treatment, one must consider if another diagnosis, such as Castlemans can be possible, Chu says. Even with all of the combined history, lab, pathology, and imaging data, the ultimate confirmation of the disease remains challenging.

• #1 Diagnosis of Multicentric Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-multicentric-castlemans-disease

  Arriving at a diagnosis of multicentric Castleman’s disease is more challenging than it may initially appear. […] The clinical symptoms of Castleman’s disease can be very diverse and can mimic almost every other condition. […] The laboratory abnormalities that are often seen accompanying Castleman’s disease would include things like anemia, thrombocytopenia, elevations in liver function tests, and certainly elevations in inflammatory biomarkers like C-reactive protein or the erythrocyte sedimentation rate. […] The symptoms that a patient presents with, the blood tests, and radiographs—usually a CT scan that show enlarged lymph nodes—all can help to bolster support for a diagnosis of Castleman’s disease, which can then be augmented by findings on histopathology. […] However, there is no pathognomonic finding on pathology, so while we all may remember Castleman’s disease from our medical textbooks as something that produces these classic looking lymph nodes with onion-skinning or other very extreme and unique histologic appearances, the fact of the matter is that there actually are a number of conditions that can cause that appearance pathologically.

• #1 Castleman Disease

  https://www.ascp.org/education/topics/castleman-disease?srsltid=AfmBOoojbAWlNvmoMUoJeE3Mdm1ogJBHgfpTwrCneaqwsBDpIPP_7Vl7

  Multicentric Castleman disease is a rare, complex disease that requires close collaboration between the pathologist, laboratory team, and clinicians to diagnose and direct therapy for optimal patient outcomes. […] ASCP is committed to helping you be equipped with the knowledge, skills, and competencies needed to ensure patients with Castleman disease are accurately diagnosed so they can receive timely, appropriate therapies to manage their disease. […] This CME/CMLE-accredited, case-based microlearning activity is designed to help laboratory team members increase their knowledge, skills, and competence in diagnosing multicentric Castleman disease and be informed of changes in clinical practice guidelines. […] Challenges in diagnosis of multicentric Castleman disease and differentiation from potential mimics

• #1 Castleman Disease in the 21st Century: An Update on Diagnosis, Assessment, and Therapy – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/july-2010/castleman-disease-in-the-21st-century-an-update-on-diagnosis-assessment-and-therapy/

  Abstract: Castleman disease (CD) is a nonclonal lymphoproliferative disorder that can affect single lymph node stations or, alternatively, can be generalized. […] Evaluation of a surgically excised lymph node is essential to render the diagnosis and to exclude malignancy and other disorders that may cause atypical lymph node hyperplasia. In the community setting, diagnosis is often delayed, and central review by an experienced pathologist is recommended in cases of suspected CD. […] Interpretation of the histopathology and subtype distinction in CD should be performed in concert with the clinical presentation and evaluation of laboratory tests for inflammatory cytokines, especially IL6, and serologic and molecular tests for HHV8, HIV, and other viruses, including Epstein-Barr virus and cytomegalovirus.

• #1 Diagnosis of Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-castlemans-disease

  The thing that is challenging about the NCCN recommendations around diagnosis of Castleman’s disease is that all of the NCCN Guidelines start with a box essentially. […] Two other important tools in making a diagnosis for Castleman’s disease are getting a piece of the involved tissue of the lymph node to make a pathologic diagnosis, and also radiography to show the extent of disease. […] My first step in making a diagnosis of Castleman’s disease is ruling out lymphoma because there’s an overlap between those two diagnoses, and lymphoma has much more severe consequences and needs to be treated much differently. […] For my money, an excisional lymph node biopsy is much preferred to a fine-needle aspirate, even though, obviously, from ease of obtaining the tissue, a fine-needle aspirate may be preferred. […] But, I think an excisional lymph node biopsy and a CT of the neck, chest, abdomen, and pelvis are really important tests in working up and diagnosing Castleman’s disease.

• #2

  https://journals.lww.com/tcmj/fulltext/2021/33010/updates_on_the_diagnosis_and_management_of.4.aspx

  Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disease. The diagnosis of this disease is typically challenging and requires collaboration between clinicians and pathologists. Moreover, it is important to exclude other diseases (such as malignancies, autoimmune diseases, and infectious diseases) that have similar clinical manifestations and pathological findings. […] The diagnosis of MCD is based on the clinical signs of systemic inflammation, serological tests, and typical pathological features. […] Given the diverse symptoms and laboratory data, lymph node biopsy is indispensable for establishing the diagnosis of MCD. The biopsies of the lymph nodes are evaluated for the histologic features, immunostaining, and special staining for pathogens. […] A definitive diagnosis of MCD should be established after excluding the above diseases.

• #2 Castleman Disease Workup: Approach Considerations, Imaging Studies, Laboratory Studies

  https://emedicine.medscape.com/article/2219018-workup

  The workup findings for Castleman disease (CD) vary among the subtypes. […] The diagnosis is made by histologic examination of an excised lymph node. […] Imaging should be performed to confirm that involvement is unicentric (ie, limited to a single lymph node or single region of enlarged lymph nodes) and to select the best lymph node for surgical excision. […] The diagnosis is made by histologic examination of an excised lymph node. […] Imaging should be performed to confirm that the patient has multiple regions of enlarged lymph nodes (ie, multicentric involvement) and to select the best lymph node for surgical excision. […] IgH gene rearrangement studies should be performed on the lymph node specimen to rule out a clonal disorder (eg, occult lymphoma). […] LANA-1 staining for HHV-8 by immunohistochemistry should be performed on all cases to confirm that HHV-8 is the pathological driver.

• #2 Updates on the diagnosis and management of multicentric Castleman disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7821823/

  The first consensus diagnostic criteria for iMCD published in 2017 comprise two major criteria and 11 minor criteria. Patients must qualify both the major criteria (typical histopathologic features of MCD in lymph nodes and enlarged lymph nodes at different lymph node stations) and at least two minor criteria (out of six abnormal laboratory findings and five clinical manifestations) to qualify the diagnostic criteria for iMCD. […] The 2015 proposed diagnostic criteria for TAFRO syndrome included the typical histopathological features of MCD in lymph nodes; negative staining for HHV-8 LANA-1; and the presence of clinical thrombocytopenia, anasarca, and organomegaly. However, lymph node biopsy is sometimes impossible to detect in patients suspected with TAFRO syndrome. Therefore, the diagnostic criteria and disease severity for TAFRO syndrome were updated in 2019, in which the histopathological features of lymph nodes are considered as a minor category, instead of a necessary histopathological criterion.

• #2 Castleman Disease: Types, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17920-castleman-disease

  Castleman disease symptoms are similar to those in other conditions, including common illnesses like the flu. Your healthcare provider may rule out these conditions first. Then, theyll perform tests to diagnose Castleman disease and determine the type. […] Tests and procedures may include: […] Lab tests. Your provider will check for abnormal blood cell counts and other microscopic signs of Castleman disease. They may perform an HIV test. A positive result is typical with HHV-8-associated MCD. […] Imaging tests. CT scans and PET scans allow your provider to locate enlarged lymph nodes in your body. They may check for other signs of Castleman disease, like an enlarged liver or spleen. […] Lymph node biopsy. A biopsy is the only way to confirm that you have Castleman disease. Your provider will examine lymph node tissue beneath a microscope to check for signs of the disease.

• #2 Diagnosis and Management of Castleman Disease- Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/features/diagnosis-and-management-of-castleman-disease/3/

  Among non-neoplastic conditions, the plasma cell variant of Castleman disease may mimic lymph nodes biopsied in the setting of rheumatoid arthritis or syphilitic (luetic) lymphadenitis due to overlapping features of follicular hyperplasia and increased interfollicular plasma cells. […] The diagnosis of rheumatoid arthritis, syphilis, or IgG4-related disease can be readily established based on clinical and laboratory features. […] For the diagnosis of other subtypes of Castleman disease in the setting of HIV infection, one should adhere to strict morphological criteria given the known histological overlap between these entities. […] The initial laboratory evaluation of patients with Castleman disease includes a complete blood count, inflammatory markers (erythrocyte sedimentation rate and C-reactive protein), complete metabolic panel, and albumin. HIV testing should be performed in all patients.

• #2 Castleman Disease Differential Diagnoses

  https://emedicine.medscape.com/article/2219018-differential

  The differential diagnosis of unicentric Castleman disease (UCD) includes other disorders that can present as a solitary enlarged lymph node and/or systemic symptoms. As a general rule, it is unusual for patients with other causes of reactive lymphadenopathy to present with enlargement of a solitary lymph node, or to have lymph node enlargement to sizes greater than 2 to 3 cm, clues that can be helpful when the histologic appearance raises the possibility of UCD. However, the „CD-like” histopathologic features can be seen in other disorders. […] Diagnosis of HHV-8negative/idiopathic multicentric Castleman disease (iMCD) requires exclusion of disorders that can mimic iMCD. In addition to the diseases listed above, these include the following: Human herpesvirus 8 (HHV-8)associated MCD – Can be excluded by negative latency-associated nuclear antigen1 (LANA-1) staining in a diagnostic lymph node.

• #2 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  Exclusion Criteria (must rule out each of these diseases that can mimic iMCD) Infection-related disorders. […] Diagnosis of disorders that have been associated with iMCD: paraneoplastic pemphigus, bronchiolitis obliterans organizing pneumonia, autoimmune cytopenias, polyneuropathy (without diagnosing POEMS), glomerular nephropathy, inflammatory myofibroblastic tumor.

• #2

  https://journals.lww.com/tcmj/fulltext/2021/33010/updates_on_the_diagnosis_and_management_of.4.aspx

  The first consensus diagnostic criteria for iMCD published in 2017 comprise two major criteria and 11 minor criteria. Patients must qualify both the major criteria (typical histopathologic features of MCD in lymph nodes and enlarged lymph nodes at different lymph node stations) and at least two minor criteria (out of six abnormal laboratory findings and five clinical manifestations) to qualify the diagnostic criteria for iMCD. […] The 2015 proposed diagnostic criteria for TAFRO syndrome included the typical histopathological features of MCD in lymph nodes; negative staining for HHV-8 LANA-1; and the presence of clinical thrombocytopenia, anasarca, and organomegaly. However, lymph node biopsy is sometimes impossible to detect in patients suspected with TAFRO syndrome. Therefore, the diagnostic criteria and disease severity for TAFRO syndrome were updated in 2019, in which the histopathological features of lymph nodes are considered as a minor category, instead of a necessary histopathological criterion. […] A definitive diagnosis is critical to improving the overall survival of these patients. Lymph node biopsy is essential to establish a definitive diagnosis and to exclude its mimics.

• #2 Castleman disease – Wikipedia

  https://en.wikipedia.org/wiki/Castleman_disease

  Castleman disease (CD) describes a group of rare lymphoproliferative disorders that involve enlarged lymph nodes, and a broad range of inflammatory symptoms and laboratory abnormalities. […] Correctly classifying the Castleman disease subtype is important, as the three subtypes vary significantly in symptoms, clinical findings, disease mechanism, treatment approach, and prognosis. […] In the United States, approximately 4,300 to 5,200 new cases are diagnosed each year. […] Surgery is considered by experts to be the first-line treatment option for all cases of UCD. […] If surgical excision is not possible, treatment is recommended for symptomatic patients. […] First-line treatment of HHV-8-associated MCD is rituximab, a drug used to eliminate a type of immune cell called the B lymphocyte.

• #2 Unicentric Castleman disease – UpToDate

  https://www.uptodate.com/contents/unicentric-castleman-disease

  Castleman disease (CD, angiofollicular lymph node hyperplasia) describes a heterogeneous group of lymphoproliferative disorders that share common histopathologic features. […] CD is classified into at least three distinct disorders that have varying clinical features, treatments, and outcomes. Classification is based on the number of regions of enlarged lymph nodes with characteristic histopathologic features and the presence/absence of human herpesvirus 8 (HHV-8, also called Kaposi sarcoma-associated herpesvirus [KSHV]) infection. […] Unicentric CD (UCD) involves one or more enlarged lymph node(s) in a single region of the body that demonstrates CD histopathologic features. […] Emerging evidence suggests that some patients with CD do not meet the criteria for UCD or MCD. The term „oligocentric CD” has been proposed for patients who have enlarged lymph nodes in two to three adjacent lymph node stations but do not meet the laboratory and clinical minor criteria required to diagnose idiopathic MCD. More research is needed, but clinical manifestations and treatment approaches for oligocentric CD appear to be more similar to UCD than MCD.

• #2 Clinicopathological comparison and therapeutic approach to Castleman disease—a case-based review – Wojtyś – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/33164/html

  Castleman disease (CD) is a rare, B-cell lymphoproliferative disorder affecting lymph nodes and extranodal anatomical locations. […] Histopathological examination remains mandatory for definitive diagnosis. […] The accuracy of histopathological examination is essential and re-evaluation has to be performed in case of relapse or unexpected course of CD. […] The CD spectrum includes several distinct diseases that were evaluated by an international group of experts affiliated with the CDCN. […] After identification of CD-like features on histopathological examination or resected tissue, the patients condition should be classified into one of the distinguishable types of CD. […] The first step is to exclude diseases that can lead to Castleman-like features on histopathology and mimic CD.

• #2 HHV-8-negative/idiopathic multicentric Castleman disease – UpToDate

  https://www.uptodate.com/contents/hhv-8-negative-idiopathic-multicentric-castleman-disease

  Emerging evidence suggests that some patients with CD do not meet the criteria for UCD or MCD. The term „oligocentric CD” has been proposed for patients who have enlarged lymph nodes in two to three adjacent lymph node stations but do not meet the laboratory and clinical minor criteria required to diagnose idiopathic MCD. More research is needed, but clinical manifestations and treatment approaches for oligocentric CD appear to be more similar to UCD than MCD.

• #2

  https://link.springer.com/article/10.1007/s00393-024-01560-5

  Due to the heterogeneous clinical symptoms, a significant proportion of patients with Castlemans disease (CD) are likely to be misdiagnosed or remain unrecognized. […] From the pathologists perspective, two prerequisites are essential for an early diagnosis: firstly, to consider the differential diagnoses of CD, and secondly, to remove a complete lymph node for optimal histological assessment. A puncture is not sufficient to be able to achieve a reliable CD diagnosis. […] If the histological findings are consistent with a CD diagnosis, a histological distinction from iMCD and the most important differential diagnoses must still be made. […] In case of clinical suspicion of Castlemans disease: procedure from the pathologists point of view.

• #2 Diagnosis of Multicentric Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-multicentric-castlemans-disease

  Patients with multicentric Castleman’s disease, in my experience, often have a delay in diagnosis from months to years. […] It can be very slow and grow slowly year after year after year. […] So, in extreme cases, that process is so slow that it may take 20 years to make a definitive diagnosis of Castleman’s disease once someone is actually prompted to really go for a full workup and biopsy and whatnot. […] It’s very variable in the amount of time that patients can present between initial symptoms and actual diagnosis.

• #2 Comprehensive analysis of 65 patients with Castleman disease in a single center in China | Scientific Reports

  https://www.nature.com/articles/s41598-022-12797-y

  Surgical removal is strongly recommended for UCD regardless of its microscopic subtype and is usually curative, but the treatment decision for MCD needs more careful consideration hinging on its specific subclassification. […] The treatment of CD in a single department is generally summarized experience, plus the rarity characteristic and clinical heterogeneity it owns, so there are diverse perceptions of this disease among doctors from different departments. […] We suggest that a multi-disciplinary team (MDT) for rare diseases, a working group made up of experts from departments of surgery, oncology, radiology, pathology, radiotherapy, etc., should be applied to propose the best treatment plan suitable for patients through a regular consultation.

• #2 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  Pathologists are essential in making this diagnosis, she said. This is not something that can be diagnosed just on the clinical side. […] An excisional biopsy is key to making the diagnosis, she said. […] If a pathologist is suspicious and maybe has a small biopsy or only a needle core biopsy, it’s appropriate to request another biopsy or to tell the ordering clinician that an entire lymph node excision is needed, she said. […] Two levels of certainty are accepted in diagnosing iMCD, and this applies to clinicians and pathologists, Dr. Neff said. You can be definitive about it if you meet all the major and minor criteria and you’ve excluded the exclusionary criteria. A diagnosis of probable iMCD can be made if the major and exclusionary criteria have been met but not the minor criteria. […] If we see it on the pathology side, even if the clinician isn’t thinking of it, it’s important that we at least mention it in our comments, Dr. Neff said, maybe provide a reference for them just to get it on their radar, so they can work up the patient appropriately.

• #2 Castleman Disease: Types, Symptoms, Causes, Complications, Diagnosis, Treatment

  https://ghealth121.com/treatments/castleman-disease/

  Diagnosis involves a thorough medical history review and physical examination, followed by several tests: […] Blood and Urine Tests: These help rule out other conditions and identify anemia or abnormalities in blood proteins. […] Imaging Tests: CT scans or PET scans are used to visualize enlarged lymph nodes and assess organ involvement. […] Lymph Node Biopsy: This essential procedure involves removing a tissue sample from an enlarged lymph node for laboratory examination, helping to confirm the diagnosis and rule out other disorders, such as lymphoma.

• #3 Signs & Symptoms – Castleman Disease Collaborative Network | CDCN

  https://cdcn.org/castleman-disease/signs-symptoms/

  Castleman disease has a broad spectrum of disease severity, ranging from mild compressive symptoms and fatigue to life-threatening multi-organ failure. […] A Castleman disease diagnosis can only be made with a lymph node biopsy that shows characteristic Castleman disease features and when all other illnesses have been evaluated and excluded. […] Symptoms in CD often overlap with symptoms of many more common illnesses. This is why a Castleman disease diagnosis cannot be made with symptoms alone and requires further testing. […] Diagnosis of CD requires an excisional lymph node biopsy that shows features of Castleman disease. […] Shows how many lymph node are affected and can help to distinguish between UCD and MCD. […] CT scan: can detect enlarged lymph nodes […] PET-CT scan: uses a radiotracer to detect enlarged lymph nodes and the activity of those lymph nodes

• #3 Castleman Disease – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK576394/

  IL-6 has proven to be a specific biomarker for this disease, contributing to its pathogenesis, symptomatology, and histopathology. However, raised blood levels of IL-6 and soluble IL-2 receptor (sIL2R), VEGF, IgA, IgE, lactate dehydrogenase, -2-microglobulin do not specifically indicate the presence of this disease but are essential for supporting its diagnosis. […] Definitive diagnosis should only be made when all other causes of lymphadenopathy are investigated and excluded. Elimination of other diseases may require biopsies, serologic or histologic studies, and clinical correlation.

• #3 Idiopathic Multicentric Castleman Disease (iMCD) – Rare Awareness Rare Education

  https://rareportal.org.au/rare-disease/idiopathic-multicentric-castleman-disease-imcd/

  Differential diagnosis (to rule out other conditions) includes unicentric Castleman disease, KSHV/HVV-8-associated Castleman disease, POEMS syndrome, Hodgkin lymphoma, non-Hodgkin lymphoma, Systemic lupus erythematous, Rheumatoid arthritis and other autoimmune diseases, and acute viral infections including EBV and HIV infection.

• #3 Think iMCD – Clinician – Suspected CD / iMCD

  https://thinkimcd.us/managing-a-patient/

  Often, the most clinically obvious sign of iMCD is two or more enlarged lymph nodes. […] A diagnosis of iMCD requires the confirmation of two or more enlarged lymph nodes AND the confirmation of histopathologic changes consistent with the CD / iMCD spectrum. […] For this reason, if it is suspected that a patient has CD / iMCD, it is essential to involve pathology services in an assessment of the lymph node (excisional biopsy recommended where possible). […] International diagnostic criteria for iMCD exist. […] These criteria are based on the principle of establishing the two major criteria, adding two or more minor criteria including at least one laboratory abnormality, and excluding potential mimics, in order to reach a diagnosis of iMCD. […] The major criteria (both must be present for a diagnosis) are: Multiple (2+) enlarged lymph nodes measuring 1 cm in short-axis diameter in multiple lymph node stations; Histopathologic lymph node features consistent with the iMCD spectrum (Requires lymph node biopsy (excisional biopsy preferred)). […] To confirm a diagnosis of iMCD: Establish both of the major criteria; Add at least 2 of 11 minor criteria, including at least 1 laboratory abnormality; Exclude all listed conditions that can mimic iMCD.

• #3 Castleman Disease | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/castleman-disease

  We know it can be confusing and scary when you cannot figure out why your child is sick, or you cannot find a treatment that works. […] Our team will review your childs medical history and do a detailed physical exam. We will also perform a variety of tests to help determine if your child has Castleman disease and to rule out other causes of enlarged lymph nodes, including: […] Blood and urine tests – These can reveal signs of inflammation, anemia, and other irregularities in your childs blood. These tests can also help rule out infections or diseases since symptoms of Castleman disease can be similar to other disorders that affect the immune system. […] Imaging tests – A CT scan or PET scan can look for enlarged lymph nodes, liver, or spleen. A PET scan can also determine if any previous treatments your child had are working. […] A lymph node biopsy This is where a tissue sample is removed from your childs lymph node and examined in our laboratory. We can then determine if your child has Castleman disease or another type of disorder, like infection or lymphoma.

• #3 SSA – POMS: DI 23022.625 – Multicentric Castleman Disease – 09/16/2020

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022625

  Multicentric Castleman Disease (MCD) is a rare disease that affects lymph nodes and other immune cell structures in the body and can severely weaken the immune system. […] Definitive diagnosis of MCD is made by biopsy or surgical resection of affected lymph nodes. In addition to nodal biopsies, CT scans, MRI, chest x-ray and/or gallium scan may aid in diagnosis. […] Lymph node biopsy with characteristic histopathology of affected lymph tissue provides the definitive diagnosis of MCD.

• #3 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20250422/New-subtype-of-Castleman-disease-identified-to-improve-diagnosis-and-treatment.aspx

  A newly identified subtype of Castleman disease will help diagnose and properly treat thousands of patients who have been caught between existing classification systems, marking the first major discovery of its kind in 45 years. […] The findings, which redefine the understanding of this rare immune disorder that affects an estimated 4,300 to 5,200 Americans, are published this week in Blood Advances by researchers from the Perelman School of Medicine at the University of Pennsylvania. […] This discovery is a game-changer for Castleman disease patients. […] Now we can match these patients-about 15 percent of all Castleman cases-with the right treatments for their specific condition. […] However, some patients’ experiences with CD don’t fit these two types, complicating diagnosis and care.

• #3 Diagnosis of Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-castlemans-disease

  A diagnosis of Castleman’s disease really begins first with suspecting it. […] The first challenge is really knowing when you should suspect it. […] In those patients, I would say anyone who presents with enlarged lymph nodes, constitutional symptoms, unexplained causes of anemia and thrombocytopenia, or high inflammatory markers, you really have to consider a diagnosis of Castleman’s disease. […] But, certainly, the diagnosis starts by suspecting it and I think that, certainly, anyone who’s had persistent symptoms of fever, night sweats, enlarged lymph nodes, other constitutional symptoms, especially in the setting of having any abnormalities in their blood counts and high inflammatory markers, those are patients that you need to suspect Castleman’s disease in. […] One way that we can facilitate providers making a prompter and accurate diagnosis of Castleman’s disease is to incorporate the disease itself in guidelines that are given to physicians for the management of many different conditions.

• #3 Castleman Disease

  https://www.ascp.org/education/topics/castleman-disease?srsltid=AfmBOoojbAWlNvmoMUoJeE3Mdm1ogJBHgfpTwrCneaqwsBDpIPP_7Vl7

  Clinical practice guidelines for diagnosis and treatment […] The importance of a multidisciplinary team-based approach to the diagnosis and management of multicentric Castleman disease. […] ASCP developed a CME/CMLE-accredited Tweetorial designed to highlight key concepts and clinical practice guidelines regarding the multidisciplinary diagnosis and treatment of patients with multicentric Castleman disease. […] This CME/CMLE-credit-bearing podcast is designed to help laboratory team members increase their knowledge, skills, and competence in diagnosing multicentric Castleman disease and be informed of changes in clinical practice guidelines.

• #3

  https://link.springer.com/article/10.1007/s00393-024-01560-5

  Due to the heterogeneous clinical symptoms, a significant proportion of patients with Castlemans disease (CD) are likely to be misdiagnosed or remain unrecognized. […] From the pathologists perspective, two prerequisites are essential for an early diagnosis: firstly, to consider the differential diagnoses of CD, and secondly, to remove a complete lymph node for optimal histological assessment. A puncture is not sufficient to be able to achieve a reliable CD diagnosis. […] If the histological findings are consistent with a CD diagnosis, a histological distinction from iMCD and the most important differential diagnoses must still be made. […] In case of clinical suspicion of Castlemans disease: procedure from the pathologists point of view.

• #4 Castleman Disease | Oncoclínicas Group Castleman Disease | Oncoclínicas Group

  https://grupooncoclinicas.com/en/tudo-sobre-o-cancer/tipos-de-cancer/castleman-disease/

  Castleman disease is a rare type of cancer that affects the lymph nodes and tissues with no known cause. […] Diagnosis of Castleman’s disease is made through biopsy. Patients often seek medical attention due to various health problems such as gastrointestinal or respiratory disorders. Physical examination or imaging typically reveals enlarged lymph nodes that can be biopsied to confirm the diagnosis. […] Therefore, the doctor will request imaging tests, such as: […] Computed tomography – to check in detail the soft tissues and whether the lymph nodes or internal organs are enlarged; […] Magnetic resonance imaging – especially if the doctor is concerned about areas near the spinal cord or brain; […] Chest X-ray – to identify if there are lymph nodes in the chest affected by the disease (usually requested when the patient presents respiratory problems as a symptom); […] Abdominal ultrasound – to identify if there are lymph nodes in the abdomen affected by the disease; and […] PET-Scan – useful for finding small growths in the lymph nodes and lymphatic tissues that do not appear on computed tomography.

• #4 Treatment guidelines (disease severity classification / disease activity index) |　Castleman disease, TAFRO, and related disease research group

  https://castleman.jp/en/introduction.html

  The following diseases should be excluded as the cause of lymphadenopathy. […] Castleman disease can present with various clinical symptoms of different degrees of severity. Frequent symptoms include low- to moderate-grade fever, general malaise, easy fatigability, weight loss, night sweats, and superficial lymphadenopathy. […] In addition to lymphadenopathy, imaging examinations may reveal hepatosplenomegaly, ascites, pleural effusion, and pulmonary interstitial shadows. […] Blood tests usually show a positive CRP and elevated serum IL-6 levels. […] In some cases, renal dysfunction, interstitial pulmonary lesions, pulmonary hypertension, dilated cardiomyopathy, autoimmune thrombocytopenia, autoimmune hemolytic anemia, endocrinopathy (such as hypothyroidism), and/or AA-amyloidosis accompany the disease.

• #4 Castleman Disease (CD) – Rare Awareness Rare Education

  https://rareportal.org.au/rare-disease/castleman-disease-cd/

  Diagnosis of Castleman Disease may be made based on the results of an excisional lymph node biopsy as well as blood and urine tests. An excisional lymph node biopsy involves the removal of the entire lymph node (either whilst awake under local anaesthetic or asleep using general aesthetic). The lymph node is then examined under a microscope to look for the characteristic changes that are associated with Castleman disease. […] Further information about the diagnostic methods for the different types of Castleman disease can be found on the individual Castleman disease pages on the RARE Portal: […] Differential diagnosis (to rule out other conditions) includes Hodgkin lymphoma, non-Hodgkin lymphoma, Systemic lupus erythematous, Rheumatoid arthritis and other autoimmune diseases, POEMS syndrome, and acute viral infections including EBV and HIV infection. Please speak to your medical team to learn more about the available diagnostic pathways for Castleman Disease.

• #4 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  Idiopathic multicentric Castleman disease, which is driven by a cytokine storm with an unknown cause, is a difficult diagnosis and one that’s often delayed, owing to the disease’s rarity and nonspecific symptoms. […] In her presentation, Dr. Neff explained that an idiopathic multicentric Castleman disease (iMCD) diagnosis must include histopathologic lymph node features consistent with the iMCD spectrum and is best assessed with an excisional lymph node biopsy. […] Diagnostic criteria developed by an international panel and published in 2017 made the diagnosis of iMCD a little more straightforward, Dr. Neff said. […] The two major criteria, both of which must be met, are multiple enlarged lymph nodes (1 cm in short-axis diameter in two or more lymph node stations) and histopathologic lymph node features consistent with the iMCD spectrum.

• #4 Castleman Disease (CD) – Causes, Symptoms, Diagnosis, Treatment, Prevention and Prognosis

  https://www.medindia.net/health/conditions/castleman-disease-rare-disease-of-lymph-nodes.htm

  If Castleman disease is suspected, your doctor will first ask you about your medical history and about the duration and severity of your signs and symptoms. Then the doctor will perform a physical examination by feeling your lymph nodes, to determine their size and consistency. The following laboratory tests may be advised to help confirm the diagnosis of CD. […] Blood and urine tests: These tests help to rule out other infections or diseases and also reveal anemia and abnormalities in blood proteins that are characteristic of Castleman disease. […] Imaging tests (CT/ MRI/ PET): They help to detect enlarged lymph nodes, liver or spleen. CT scan or MRI of your neck, chest, abdomen and pelvis may be performed. Positron emission tomography (PET) scans may also be used to diagnose Castleman disease and to assess the effectiveness of treatment.

• #4 Diagnosis of Multicentric Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-multicentric-castlemans-disease

  Arriving at a diagnosis of multicentric Castleman’s disease is more challenging than it may initially appear. […] The clinical symptoms of Castleman’s disease can be very diverse and can mimic almost every other condition. […] The laboratory abnormalities that are often seen accompanying Castleman’s disease would include things like anemia, thrombocytopenia, elevations in liver function tests, and certainly elevations in inflammatory biomarkers like C-reactive protein or the erythrocyte sedimentation rate. […] The symptoms that a patient presents with, the blood tests, and radiographs—usually a CT scan that show enlarged lymph nodes—all can help to bolster support for a diagnosis of Castleman’s disease, which can then be augmented by findings on histopathology. […] However, there is no pathognomonic finding on pathology, so while we all may remember Castleman’s disease from our medical textbooks as something that produces these classic looking lymph nodes with onion-skinning or other very extreme and unique histologic appearances, the fact of the matter is that there actually are a number of conditions that can cause that appearance pathologically.

• #4 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20250422/New-subtype-of-Castleman-disease-identified-to-improve-diagnosis-and-treatment.aspx

  The study found that OligoCD patients exhibit fewer and less severe symptoms than those with iMCD, suggesting that surgical removal of affected lymph nodes-effective for the milder UCD-may be more appropriate than the intense treatments used for iMCD. […] However, the team emphasizes the need for further research to refine treatment guidelines and a further understanding of how OligoCD develops, with ACCELERATE poised to provide ongoing insights. […] The Penn Clinical and Research teams also worked with several patients from the Castleman Disease Collaborative Network (CDCN) who shared their experiences, which helped identify the gaps in existing classifications and inspired the push to define OligoCD as a new subtype.

• #4 How iMCD Is Diagnosed | SYLVANT® (siltuximab)

  https://sylvant.com/understanding-imcd/diagnosis/

  Getting to an iMCD diagnosis starts with ruling out other conditions. Because iMCD can look like cancer, infectious diseases, or autoimmune conditions, your healthcare team will evaluate you or your loved one for more than 15 other conditions before reaching a diagnosis. […] My doctor [did] all kinds of tests—CT scan, ultrasound, and of course, blood tests. […] The road to diagnosis can be long. That’s why it’s important for you to report every symptom to your healthcare team. You’ll typically start by meeting with your primary care physician who may refer you to a specialist. […] Tests you may undergo include physical examination of swollen lymph nodes, enlarged organs, and fluid accumulation, blood tests to look for signs of infection, autoimmune conditions, and kidney damage, and imaging tests like an x-ray, a positron emission tomography (PET) scan, or a computed tomography (CT) scan to reveal the presence of multiple swollen lymph nodes and enlarged organs. […] An excisional biopsy removes the entire lymph node and is a more effective way to diagnose iMCD. […] Getting an accurate diagnosis for a rare disease like iMCD is never easy. That’s why it’s important to work with your doctors and remain persistent throughout the diagnostic journey.

• #4

  https://haematologica.org/article/view/haematol.2023.283603

  Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. […] Making a diagnosis of iMCD is challenging because it is based on non-specific clinical features and characteristic lymph node histopathology. […] Underdiagnosis is likely as diagnostic criteria were not developed until 2017, and there is no known diagnostic serum biomarker. […] Data from the USA before 2012 suggested 5- and 10-year mortality rates of 35% and 60% for iMCD patients whereas more recent data from electronic medical records suggest 25% mortality at 5 years. […] We found that iMCD-TAFRO patients face a significant hospitalization burden, requiring more time in the hospital than iMCDNOS patients during the year surrounding diagnosis (median [interquartile range]: 36 [18-61] days vs. 0 [0-4] days; P0.001).

• #5 Emerging role of 18F-FDG PET/CT in Castleman disease: a review | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-00963-1

  Castleman disease (CD) requires more robust characterization, earlier diagnosis, and an accurate tool for both monitoring and treatment response evaluation; FDG-PET/CT is particularly suited for this. […] The standard investigative workup in Castleman disease usually begins with lymph node biopsy followed by radiological investigation with PET/CT preferred, complete blood count, serum chemistry, markers of inflammation, serum cytokine levels, viral serology for HHV-8 and HIV, and protein electrophoresis, immunofixation, and quantitative immunoglobulins. […] A formal diagnostic criteria have only been established for iMCD and are summarized in Table 1. […] Although excisional lymph node biopsy is the only way to definitively diagnose CD based on its histology, existing evidence suggests that FDG-PET/CT should be performed beforehand to help determine CD subtype, consider the possibility of lymphoma, and identify ideal lesions for biopsy.

• #5 Diagnosis of Multicentric Castleman’s Disease

  https://www.targetedonc.com/view/diagnosis-of-multicentric-castlemans-disease

  That becomes much more specific when it’s added to the clinical signs and symptoms that a patient presents with. […] There are certainly things that, in my experience, help to solidify that diagnosis. […] I would say that oftentimes, clinicians will look for evidence for elevated interleukin-6 in the plasma, and I think that has a very good positive predictive value. […] If it’s very elevated, then I think chances are likely that you could have Castleman’s disease. […] But it has a poor negative predictive value. […] We know that there are patients in whom you can’t detect interleukin-6 in the peripheral blood but still have the disease, so it’s not a perfect test. […] The gold standard would be the detection of human herpesvirus-8 in the lymph node by immunohistochemistry. […] But there are other ways you can solidify that diagnosis, like quantifying the virus from the plasma in the peripheral blood with a polymerase chain reaction.

• #5 Idiopathic multicentric Castleman disease – CAP TODAY

  https://www.captodayonline.com/idiopathic-multicentric-castleman-disease/

  Pathologists are essential in making this diagnosis, she said. This is not something that can be diagnosed just on the clinical side. […] An excisional biopsy is key to making the diagnosis, she said. […] If a pathologist is suspicious and maybe has a small biopsy or only a needle core biopsy, it’s appropriate to request another biopsy or to tell the ordering clinician that an entire lymph node excision is needed, she said. […] Two levels of certainty are accepted in diagnosing iMCD, and this applies to clinicians and pathologists, Dr. Neff said. You can be definitive about it if you meet all the major and minor criteria and you’ve excluded the exclusionary criteria. A diagnosis of probable iMCD can be made if the major and exclusionary criteria have been met but not the minor criteria. […] If we see it on the pathology side, even if the clinician isn’t thinking of it, it’s important that we at least mention it in our comments, Dr. Neff said, maybe provide a reference for them just to get it on their radar, so they can work up the patient appropriately.

• #5 Castleman Disease in the 21st Century: An Update on Diagnosis, Assessment, and Therapy – Hematology & Oncology

  https://www.hematologyandoncology.net/archives/july-2010/castleman-disease-in-the-21st-century-an-update-on-diagnosis-assessment-and-therapy/

  Laboratory findings at diagnosis include anemia, elevated ESR, C-reactive protein (CRP), IL6, fibrinogen, proteinuria, abnormal thyroid function tests, hypergammaglobulinemia, and thrombocytosis. A significant number of patients have elevated plasma VEGF levels. […] Imaging by computed tomography scan is useful to distinguish unicentric from multicentric disease and to detect hepatosplenomegaly, pleural effusion, and ascites. […] There have been no published randomized clinical trials regarding the management of CD. Most of the literature is confined to small series or case reports, and it is difficult to make firm recommendations. […] The preferred management of unicentric CD is complete surgical excision, which is curative in approximately 95% of patients and affords resolution of constitutional symptoms, if present.

• #5 Castleman disease – Wikipedia

  https://en.wikipedia.org/wiki/Castleman_disease

  First line treatment for iMCD is anti-IL-6 therapy with siltuximab (or tocilizumab, if siltuximab is not available). […] The clinical utility of subtyping Castleman disease by histologic features is uncertain, as histologic subtypes do not consistently predict disease severity or treatment response. […] Guidelines recommend against using histologic subtype to guide treatment decisions. […] In 2016, formal diagnostic criteria and definition of the disease was established, which will allow for better understanding and the ability to appropriately track and research CD. […] In 2017, international consensus diagnostic criteria for idiopathic multicentric Castleman disease (iMCD) were established for the first time. […] In 2020 the first evidence based diagnostic criteria and treatment guidelines were established for unicentric Castleman disease.

• #5 Emerging role of 18F-FDG PET/CT in Castleman disease: a review | Insights into Imaging | Full Text

  https://insightsimaging.springeropen.com/articles/10.1186/s13244-021-00963-1

  Beyond identifying lymphadenopathy, FDG-PET/CT can be utilized to monitor response to treatment. […] Therefore, the role of FDG-PET/CT as both a prognostic and a monitoring tool should be further investigated. […] In the future, we believe that FDG-PET/CT and associated techniques will be useful in the diagnosis and categorization of CD, in differentiation between mimicking conditions, and monitoring of disease progression and response to treatment.

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