Materiały źródłowe

• #1 Cryoglobulinemia – Symptoms, diagnosis and treatment | BMJ Best Practice US

  https://bestpractice.bmj.com/topics/en-us/870

  Cryoglobulinemia may be asymptomatic or present acutely. […] Diagnosis is based on clinical and laboratory findings. Leukocytoclastic vasculitis is the histologic hallmark of MC. […] Key diagnostic factors include arthralgia, weakness, vasculitis, and lower-extremity vascular purpura. […] 1st tests to order include fasting qualitative serum cryoglobulins, CBC, comprehensive chemistry panel, erythrocyte sedimentation rate, CRP, urinalysis, ECG, CXR, rheumatoid factor, complement CH50, C3, C4, hepatitis C virus (HCV) antibody, and hepatitis B virus antibody. […] Tests to consider include HIV antibody, antinuclear antibody (ANA), Sjogren syndrome antigens, extractable nuclear antigen (ENA) antibody, and skin biopsy.

• #1 Cryoglobulinemia – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cryoglobulinemia/diagnosis-treatment/drc-20449756

  Diagnosis of cryoglobulinemia involves a blood test. The blood is kept at body temperature, 98.6 F (37 C), for a time. Then it’s cooled before being tested. The sample must be handled this way to get correct results. […] Other blood and urine tests also might be used to find the underlying cause.

• #1 Cryoglobulinemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557606/

  Cryoglobulinemia is a rare medical condition characterized by the presence of abnormal proteins called cryoglobulins in the blood, which precipitate or clump together at low temperatures. […] Diagnosis involves measuring cryoglobulin levels along with low C4 complement levels. […] The most predictive measure for cryoglobulinemia is the measurement of cryoglobulin coupled with a low C4 complement level. This combination is typical of cryoglobulinemia syndromes. […] In the lab detection of cryoglobulins, approximately 10 to 20 mL of blood is collected and prepared at 37 C without the addition of anticoagulants. […] A cryocrit is defined as the percentage of cryoglobulin compared to the total serum volume read from a calibrated sedimentation tube. […] Another common method of evaluating cryoglobulinemia is immunochemical analysis.

• #1 Cryoglobulinemia: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329255-overview

  Cryoglobulinemia is characterized by the presence of cryoglobulins in the serum. This may result in a clinical syndrome of systemic inflammation (most commonly affecting the kidneys and skin) caused by cryoglobulin-containing immune complexes. […] Cryoglobulinemia may be classified based on cryoglobulin composition with the Brouet classification, which is as follows: Type I cryoglobulinemia, or simple cryoglobulinemia, is the result of a monoclonal immunoglobulin, usually immunoglobulin M (IgM) or, less frequently, immunoglobulin G (IgG), immunoglobulin A (IgA), or light chains. […] Types II and III cryoglobulinemia (mixed cryoglobulinemia) contain rheumatoid factors (RFs), which are usually IgM and, rarely, IgG or IgA. These RFs form complexes with the fragment, crystallizable (Fc) portion of polyclonal IgG.

• #1 Final Diagnosis — Case 593

  https://path.upmc.edu/cases/case593/dx.html

  The concentration of a cryoglobulin can be measured as a cryocrit. Although this is a convenient, rapid and an inexpensive method it is not standardized as to volume, tube size, or conditions for centrifugation, and should not be considered an index of disease activity when comparing different patients. […] Immunofixation-Immunofixation is currently the method of choice for the typing of cryoglobulins into type I, II and III. […] Type II cryoglobulins are mixed polyclonal and monoclonal immunoglobulins, frequently found in excess of 1 mg/mL. They may begin to precipitate within hours of refrigeration and are usually apparent by the next day. Our case is an example of this type of cryoglobulinemia. […] Other tests helpful in completing the picture of cryoglobulinemia include serum viscosity, complement levels (C3, C4 and total hemolytic complement CH50) and rheumatoid factor.

• #1 Cryoglobulinaemia – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/870

  Cryoglobulinaemia may be asymptomatic or present acutely. […] Diagnosis is based on clinical and laboratory findings. Leukocytoclastic vasculitis is the histological hallmark of MC. […] Key diagnostic factors include presence of risk factors, arthralgia, weakness, vasculitis, and lower-extremity vascular purpura. […] 1st investigations to order include fasting qualitative serum cryoglobulins, FBC, comprehensive blood chemistry profile, erythrocyte sedimentation rate, CRP, urinalysis, ECG, CXR, rheumatoid factor, complement CH50, C3, C4, hepatitis C virus (HCV) antibody, and hepatitis B virus antibody. […] Investigations to consider include HIV antibody, antinuclear antibody (ANA), Sjogren’s syndrome antigens, extractable nuclear antigen (ENA) antibody, and skin biopsy.

• #1 Cryoglobulinemia Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/329255-workup

  To evaluate for serum cryoglobulins, the blood specimen must be collected in warm tubes (37C) in the absence of anticoagulants. The blood sample must be allowed to clot before removal of serum with centrifugation (at 37C). […] Repeat centrifugation is performed to determine cryocrit (volume of precipitate as a percentage of original serum volume). Cryoglobulin concentration may be determined via spectrophotometric analysis. Specific immunologic assays may be used to identify cryoglobulin components (immunoglobulins, light chains, clonality). […] Hepatitis C virus (HCV) testing is required. HCV antibody or HCV RNA testing may be diagnostic. If HCV test results are negative and clinical suspicion remains high, these tests may be performed on the cryoprecipitate. […] Tissue biopsy may be required for diagnosis when patients with vasculitis, renal disease, or both are evaluated.

• #1 The diagnosis and classification of the cryoglobulinemic syndrome – PubMed

  https://pubmed.ncbi.nlm.nih.gov/24424176/

  Cryoglobulinemia refers to the presence of reversible precipitation of immunoglobulins in the blood upon exposure to reduced (body) temperature. […] In clinical practice, the detection and typing of cryoglobulins appear to be poorly standardized. A consensus protocol for detection and typing of cryoglobulins, as extracted from the literature, is presented. […] Only for the latter situation, i.e. cryoglobulinemic vasculitis in the presence of mixed-type cryoglobulinemia, preliminary classification criteria have been defined. Besides the presence of cryoglobulins, at least two of three items have to be present. These items include a restricted number of self-reported clinical history, typical clinical manifestations, and specified laboratory findings. These classification criteria await further validation in an independent patient cohort.

• #1 Quantitative vs. Qualitative Cryoglobulinemia Testing and a Second Positive Cryo Test – Alliance for Cryoglobulinemia

  https://allianceforcryo.org/quantitative-vs-qualitative/

  Qualitative vs Quantitative testing for Cryoglobulins. A single qualitative positive test for cryoglobulins qualifies as a diagnosis for cryoglobulinemia. […] The level of cryoglobulins in the blood are NOT good indicators for the severity or diagnosis of cryoglobulinemia. […] Cryoglobulinemia is defined as the presence of cryoglobulins in the blood. Because of this clear and simple definition, the mere presence of cryoglobulins is taken as the diagnostic indicator for cryoglobulinemia. If you have cryoglobulins, regardless of the level, you have cryoglobulinemia, by definition. […] Some doctors like to have a second positive for cryogobulins to confirm the diagnosis. This approach is likely not workable most of the time unless you can find a facility that does the test themselves, does it correctly, does it reliably, and you don’t mind repeating the test *A LOT*. […] In very real terms, one positive in the face of symptoms for cryo is compelling, and this is where most doctors will make the call, and call it cryoglobulinemia.

• #1 RheumaKnowledgy » Cryoglobulinemia

  https://www.rheumaknowledgy.com/cyroglobulinemia/

  Differential Diagnosis: Conditions that may mimic cryoglobulinemia include vasculitis (e.g., polyarteritis nodosa, Henoch-Schnlein purpura), SLE, and lymphoma. Henoch-Schnlein purpura is more likely to be seen in young adults or children. Isolated renal involvement may occur with cryoglobulinemia and often requires renal biopsy for definitive diagnosis and exclusion of other forms of glomerulonephritis. […] Therapy: If an underlying disorder is identified, therapy should be aimed at that process. Hepatitis C may be treated with IFN-a/ribavirin. Acute complications of the cryoglobulins themselves, such as rapidly progressive renal failure or symptoms associated with hyperviscosity, should be treated with a course of intensive plasmapheresis. Cyclophosphamide and steroids are used to treat patients with renal or nervous system involvement.

• #1 Identification of Key Predictors of Cryoglobulinemia Severity at Diagnosis: Threshold, Type, and Severity Score at Diagnosis

  https://www.mdpi.com/2077-0383/14/2/556

  Identification of Key Predictors of Cryoglobulinemia Severity at Diagnosis: Threshold, Type, and Severity Score at Diagnosis […] Understanding the presentation of cryoglobulinemia-related symptoms based on cryoprecipitate levels, GC type, and severity at diagnosis is essential for effective management. […] This study identifies severity markers in patients with positive cryoprecipitate and proposes a score related to severity at diagnosis. […] Factors influencing disease severity include the type of cryoglobulin (type I or mixed), cryoprecipitate levels, the presence of underlying conditions (e.g., hematological malignancy, autoimmune disorders, chronic infection), and multi-organ involvement. […] Cryoprecipitate levels exceeding 50 mg/L are considered clinically significant, but patients with lower levels can also experience severe, life-threatening complications.

• #1 Study Looks at Cryoglobulin Detection & Immunological Characteristics – The Rheumatologist

  https://www.the-rheumatologist.org/article/study-looks-at-cryoglobulin-detection-immunological-characteristics/

  A study that focused on the detection and immunological characteristics of cryoglobulins provides insights for rheumatologists and other rheumatology providers, as well as lab professionals. […] The researchers analyzed cryoglobulin isotype, clonality, concentration and rheumatoid factor in cryoprecipitate, as well as serum complement and rheumatoid factor. […] If no cryoprecipitates were detected, one or two blood samples were analyzed to confirm the negative result. […] Cryoglobulins were identified by electrophoresis-immunofixation of the dissolved cryoprecipitate with various antisera and were then classified by their immunoglobulin clonality profile, according to the classification of cryoglobulin (type I: monoclonal Ig; type II: monoclonal Ig with rheumatoid factor activity; type III: polyclonal Ig with rheumatoid factor activity).

• #1 Study Looks at Cryoglobulin Detection & Immunological Characteristics – The Rheumatologist

  https://www.the-rheumatologist.org/article/study-looks-at-cryoglobulin-detection-immunological-characteristics/

  The study also found that complement activation by immune complexes specifically, IgM cyroglobulin associated with IgG is one of the mechanisms contributing to cryoglobulinemic vasculitis, especially hepatitis C-associated cryoglobulinemic vasculitis. […] Improvement of techniques used to detect rheumatoid factor in a cryoprecipitate is needed, the researchers note in the study.

• #1 Shining a Warm Light on Cryoglobulinemia

  https://www.cancernetwork.com/view/shining-warm-light-cryoglobulinemia

  We recommend screening for cryoglobulinemia in all patients with HCV infection, livedo reticularis, vasculitic cutaneous ulcers, positive rheumatoid factor or rheumatoid vasculitis, membranoproliferative glomerulonephritis, or atypical Waldenstrm macroglobulinemia. […] When testing for cryoglobulinemia, neither serum protein electrophoresis (SPEP) nor serum immunofixation (IF) are adequate screening tests. […] Thus serum cooling studies are required to diagnose or rule out cryoglobulinemia; there are no other laboratory findings sensitive and specific enough to establish the diagnosis. […] Therefore-and to distinguish it from overt WM-cryoglobulinemia is recognized as a variant of WM and is classified as an IgM-related disorder, along with cold agglutinin disease, amyloid light-chain (AL) amyloidosis, and IgM neuropathies.

• #1 Shining a Warm Light on Cryoglobulinemia

  https://www.cancernetwork.com/view/shining-warm-light-cryoglobulinemia

  When cryoglobulins are detected in a patient without HCV infection or other apparent etiology, we recommend thorough staging studies to rule out possible lymphoma. […] If treatment is indicated for symptomatic disease, then we agree with Dr. Ramos-Casals and colleagues that treatment should be directed at the underlying cause. […] For refractory disease we continue to use chemotherapy, usually cyclophosphamide and high-dose corticosteroids or fludarabine-based therapy. […] If a patient has life-threatening vasculitis or renal failure, we continue to employ plasmapheresis to acutely lower the cryocrit while initiating high-dose corticosteroids, cyclophosphamide, and anti-CD20 therapy. […] Importantly, anti-CD20 therapy with rituximab has been reported in HCV-positive cryoglobulinemia without clinically significant exacerbations of the HCV infection. […] Although superficially puzzling, this finding is consistent with the conception of cryoglobulinemia as a variant of WM, which is to say a low-grade lymphoma that, once established, should persist even after the inducing stimulus has been removed.

• #1 RheumaKnowledgy » Cryoglobulinemia

  https://www.rheumaknowledgy.com/cyroglobulinemia/

  Surgery: Surgery is only used for diagnostic biopsy such as kidney or nerve. […] Prognosis: In the absence of renal or neurologic involvement, many patients remain stable for years. Rapidly progressive glomerulonephritis is associated with a poorer prognosis. All patients should be monitored for transformation to neoplastic disease.

• #1

  https://journals.lww.com/ajg/fulltext/2017/10001/new_onset_cryoglobulinemia_following_orthotopic.2283.aspx

  Hepatitis C (HCV) accounts for up to 90% of all mixed-cryoglobulinemia (MC). Direct acting antiviral (DAA) medications decrease cryoglobulin levels in 89% of patients. […] In patients with HCV who achieve SVR, up to 52% patients may still have persistent cryoglogulenemia after twelve weeks of DAA and may even have relapses of cryoglobulinemia despite SVR. This case is novel in that the patient had no diagnosis of cryoglobulenemia prior to liver transplantation and HCV treatment. Our patient developed renal failure, leukocytoclastic vasculitis, and acute systolic heart failure as a result of cryoglobulinemia despite SVR.

• #1 How do I Deal with Cryoglobulinemia?

  https://www.iomcworld.com/open-access/how-do-i-deal-with-cryoglobulinemia-111971.html

  The presence of cryoglobulins in the serum distinguishes cryoglobulinemia from other diseases. […] Cryoglobulinemia is diagnosed by looking for common clinical signs and symptoms in the presence of cryoglobulins in the blood. […] To minimize precipitation, samples should be moved and centrifuged at 37°C before serum extraction. […] If the test for cryoglobulin is negative but the index of suspicion remains high, the test should be repeated after contacting the laboratory to check that the sample was handled properly. […] Overall, cryoconite should be used just for diagnosis because the cryoconite and treatment response have a weak relationship.

• #2 Cryoglobulinemia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK557606/

  Cryoglobulinemia is a rare medical condition characterized by the presence of abnormal proteins called cryoglobulins in the blood, which precipitate or clump together at low temperatures. […] Diagnosis involves measuring cryoglobulin levels along with low C4 complement levels. […] The most predictive measure for cryoglobulinemia is the measurement of cryoglobulin coupled with a low C4 complement level. This combination is typical of cryoglobulinemia syndromes. […] In the lab detection of cryoglobulins, approximately 10 to 20 mL of blood is collected and prepared at 37 C without the addition of anticoagulants. […] A cryocrit is defined as the percentage of cryoglobulin compared to the total serum volume read from a calibrated sedimentation tube. […] Another common method of evaluating cryoglobulinemia is immunochemical analysis.

• #2 Cryoglobulinemia Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/329255-workup

  To evaluate for serum cryoglobulins, the blood specimen must be collected in warm tubes (37C) in the absence of anticoagulants. The blood sample must be allowed to clot before removal of serum with centrifugation (at 37C). […] Repeat centrifugation is performed to determine cryocrit (volume of precipitate as a percentage of original serum volume). Cryoglobulin concentration may be determined via spectrophotometric analysis. Specific immunologic assays may be used to identify cryoglobulin components (immunoglobulins, light chains, clonality). […] Hepatitis C virus (HCV) testing is required. HCV antibody or HCV RNA testing may be diagnostic. If HCV test results are negative and clinical suspicion remains high, these tests may be performed on the cryoprecipitate. […] Tissue biopsy may be required for diagnosis when patients with vasculitis, renal disease, or both are evaluated.

• #2 Cryoglobulinemia | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/20147

  Cryoglobulins are proteins that precipitate from an individual’s serum or plasma at temperatures lower than 37 C. Diagnosis of this entity should be suspected in patients presenting with skin ulcers, arthralgia, glomerulonephritis, neuropathy, and purpura. […] The most predictive measure for cryoglobulinemia is the measurement of cryoglobulin coupled with a low C4 complement level. This combination is typical of cryoglobulinemia syndromes. […] In the lab detection of cryoglobulins, approximately 10 to 20 mL of blood is collected and prepared at 37 C without the addition of anticoagulants. […] A cryocrit is defined as the percentage of cryoglobulin compared to the total serum volume read from a calibrated sedimentation tube. […] Another common method of evaluating cryoglobulinemia is immunochemical analysis.

• #2 What Is Cryoglobulinemia?

  https://www.icliniq.com/articles/blood-health/cryoglobulinemia

  Results: Type I Cryoglobulinemia – Will exhibit precipitation of the cryoglobulin proteins within 24 hours. It may also take a window of 3 to 5 days. […] Type II and Type III Cryoglobulinemia – They will present the precipitate within 5 to 7 days after initial refrigeration of the patients serum. […] The cryoglobulin values in the laboratory are determined as follows: In individuals without cryoglobulinemia, the cryocrit value is usually close to zero. However, a value 0.5 to 1 percent is considered markedly significant. […] In type II Cryoglobulinemia the cryocrit value usually falls between 2 to 7 percent. […] In type III Cryoglobulinemia, the cryocrit value usually falls within 1 to 3 percent. […] However, there is no established correlation between the calculated laboratory cryocrit levels and clinical manifestations.

• #2 Cryoglobulinemia – UF Health

  https://ufhealth.org/conditions-and-treatments/cryoglobulinemia

  Cryoglobulinemia is the presence of abnormal proteins in the blood. These proteins thicken in cold temperatures. […] Tests for cryoglobulinemia include: Complete blood count (CBC). Complement assay — complement levels will be low. Cryoglobulin test — may show presence of cryoglobulins. (This is a complicated laboratory procedure that involves many steps. It is important that the lab that performs the test is familiar with the process.) Liver function tests — may be high if hepatitis C is present. Rheumatoid factor — positive in types II and III. Skin biopsy — may show inflammation in blood vessels, vasculitis. Protein electrophoresis – blood — may show an abnormal antibody protein. Urinalysis — may show blood in the urine if the kidneys are affected. […] Current direct-acting medicines for hepatitis C eliminate the virus in nearly all people. As hepatitis C goes away, the cryoglobulins will disappear in about one half of all people over the next 12 months. Your provider will continue to monitor the cryoglobulins after treatment.

• #2 Cryoglobulinemic Vasculitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/cryoglobulinemic-vasculitis/

  Cryoglobulinemic vasculitis is a rare form of vasculitis, a family of rare disorders characterized by inflammation of the blood vessels, which can restrict blood flow and damage vital organs and tissues. […] In diagnosing cryoglobulinemic vasculitis, your doctor will consider a number of factors including a detailed medical history and physical examination, as well as: […] Blood tests: to detect the presence and type of cryoglobulins in the blood, hepatitis C virus, and the presence of abnormal blood cell counts […] Urinalysis: to look for blood and protein in the urine, which can indicate kidney involvement […] Imaging studies: chest X-ray, computed tomography (CT) scans of the lungs, magnetic resonance angiography (MRA), computed tomography angiography (CTA), or angiograms (images of the arteries), if indicated

• #2 Cryoglobulinemia Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/329255-workup

  Histologic evaluation of affected lung, kidney, and muscle reveals eosinophilic material in the lumen of small vessels with frequent extension into the vessel intima and inflammation of the vessel wall. […] Although biopsy samples generally exhibit inflammatory vascular changes (eg, leukocytoclastic vasculitis in patients with vasculitic purpura), intraluminal cryoglobulin deposits may be observed, especially in renal glomeruli.

• #2 Essential Cryoglobulinemia Diagnosis | Medical Algorithm | Medicalalgorithms.com

  https://www.medicalalgorithms.com/cryoglobulinemia-diagnosis

  Criteria for the diagnosis of essential cryoglobulinemic vasculitis were proposed at an International Consensus Conference held at the University of North Carolina, Chapel Hill, North Carolina. […] Features: […] (1) cryoglobulin deposits in the vascular lesions […] (2) cryoglobulin in the serum […] (3) glomerulonephritis […] (4) skin involvement […] Objective: clinical diagnosis, including family history for genetics, laboratory tests, criteria for diagnosis […] ICD-10: D89.1 – Cryoglobulinaemia.

• #2 Final Diagnosis — Case 593

  https://path.upmc.edu/cases/case593/dx.html

  The most important variable in cryoglobulin testing is maintaining the temperature at 37C while drawing the sample, its transportation and separation of serum from other blood elements. […] A cryoglobulin screen may be falsely positive, and immunochemical characterization of any cryoprecipitate obtained is necessary to confirm the presence of immunoglobulin. […] There may be considerable changes in the composition of mixed cryocomplexes over time as IgM levels fluctuate with activity of disease, decrease with treatment, or if patients become hypogammaglobulinemic. […] An association between HCV infection and the syndrome of purpura, arthralgias, renal disease, and neuropathy was first reported in 1990 and accounts for approximately 60% to 80% of cases of mixed cryoglobulinemia. […] First-line therapy for mixed cryoglobulinemia due to HCV infection is antiviral therapy with pegylated interferon-? and ribavirin. Viral eradication usually produces marked reduction of complications and arrests end organ damage along with the clearance of cryoglobulins.

• #2 Cryoglobulinemia – Wikipedia

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

  Cryoglobulinemia and cryoglobulinemic disease must be distinguished from cryofibrinogenemia or cryofibrinogenemic disease, conditions that involve the cold-induced intravascular deposition of circulating native fibrinogens. […] Rheumatoid factor is a sensitive test for cryoglobulinemia. The precipitated cryoglobulins are examined by immunoelectrophoresis and immunofixation to detect and quantify the presence of monoclonal IgG, IgM, IgA, light chain, or light chain immunoglobins. Other routine tests include measuring blood levels of rheumatoid factor activity, complement C4, other complement components, and hepatitis C antigen. […] In all events, further studies to determine the presence of hematological, infections, and autoimmune disorders are conducted on the basis of these findings as well as each case’s clinical findings.

• #2 Identification of Key Predictors of Cryoglobulinemia Severity at Diagnosis: Threshold, Type, and Severity Score at Diagnosis

  https://www.mdpi.com/2077-0383/14/2/556

  Treatment strategies are tailored according to disease severity, CG type, and the presence of underlying conditions. […] The study sheds light on the clinical presentations associated with CG types. […] Patients with severe disease exhibited distinctive clinical features including purpura, Raynaud’s phenomenon, and leukocytoclastic vasculitis on skin biopsy. […] The study’s most innovative contribution is the development of a clinically applicable severity scoring system. […] A novel severity scoring system based on four items (purpura, Raynaud’s phenomenon, hematological conditions, and cryoprecipitate) may predict severity and clinical decision-making and patient care.

• #2 Study Looks at Cryoglobulin Detection & Immunological Characteristics – The Rheumatologist

  https://www.the-rheumatologist.org/article/study-looks-at-cryoglobulin-detection-immunological-characteristics/

  The study also found that complement activation by immune complexes specifically, IgM cyroglobulin associated with IgG is one of the mechanisms contributing to cryoglobulinemic vasculitis, especially hepatitis C-associated cryoglobulinemic vasculitis. […] Improvement of techniques used to detect rheumatoid factor in a cryoprecipitate is needed, the researchers note in the study.

• #2 Final Diagnosis — Case 593

  https://path.upmc.edu/cases/case593/dx.html

  FINAL DIAGNOSIS Type II mixed cryoglobulinemia secondary to chronic HCV infection, Membranoproliferative glomerulonephritis secondary to cryoglobulinemia. […] Cryoglobulin testing should be carried out if the patient has clinical or laboratory features suggestive of presence of cryoglobulins (Severe cold-dependent symptomatology, Raynaud’s syndrome, acrocyanosis of the digits or helices of the ears, gangrene in the absence of other known causes of vascular occlusion, livedoid vasculitis, gelling of blood on blood drawing, pathologic evidence of occlusive vasculopathy due to immunoglobulins, syndrome of cutaneous vasculitis-purpura/leg ulcers, arthralgias/arthritis, Immune-complex nephritis, Neuropathy, renal disease characterized pathologically by membranoproliferative glomerulonephritis, hyaline inclusions in glomeruli, vasculitis, chronic hepatitis with extrahepatic clinical manifestations, high-titer rheumatoid factor activity in the absence of clear-cut rheumatic disease, strikingly depressed levels of C4, Extra-articular manifestations of rheumatoid arthritis, Chronic inflammatory liver diseases, chronic inflammatory bowel diseases) or is known to have a disease that may be associated with cryoglobulins (Multiple myeloma, Waldenstrom’s macroglobulinemia, chronic hepatitis C virus infection, Sjogren’s syndrome, Chronic lymphocytic leukemia, Non-Hodgkins’ lymphoma, cold agglutinin disease, chronic infections, autoimmune diseases, Systemic lupus erythematosus, Rheumatoid arthritis, Inflammatory bowel diseases, Biliary cirrhosis).

• #2 Cryoglobulinemia: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/329255-overview

  Some of the sequelae of cryoglobulinemia are thought to be related to immune-complex disease (eg, glomerulonephritis, chronic vasculitis), but not all persons with cryoglobulinemia present with these manifestations. […] The prognosis in these patients depends on the presence of underlying diseases (eg, lymphoproliferative disorders, hepatitis B or C infection, connective-tissue disease), all of which increase the mortality rate over that of the healthy population and more accurately direct estimates of individual survival. […] For example, the prognosis in patients with chronic hepatitis C depends on their response to treatment; manifested by their decrease in viral load. […] Complete or partial remission of the manifestations of mixed cryoglobulinemic vasculitis, with high, sustained viral response rates, have been reported in more than 80% of patients with chronic HCV infection taking direct-acting viral agents. […] Morbidity due specifically to cryoglobulinemia may be significant, with infection and cardiovascular disease being major considerations.

• #2 Relevance and diagnostics of cryoproteins

  https://www.degruyter.com/document/doi/10.1515/labmed-2015-0048/html?lang=en

  Conversely, the positive detection of cryoproteins is a laboratory finding that must trigger further analysis of the conditions described below, if they are not already known. […] Cryoglobulins are immunoglobulins (antibodies) in human serum, which at reduced temperature (37 C) precipitate spontaneously, and usually dissolve when heated again. […] Cryofibrinogen is an insoluble (when it is cold) clot in human EDTA plasma made up of fibrinogen, fibrin, fibrin degradation products, thrombin, immunoglobulins, albumin and other proteins that forms at 4 C and usually dissolves again at 37 C. […] Cryofibrinogenemia is the underdiagnosed increase of a cryoprotein, which can be life threatening. […] The correct blood collection in connection with a suspected cryoproteinemia is done via blood collection systems pre-heated to at least 37 C.

• #3 What Is Cryoglobulinemia?

  https://www.icliniq.com/articles/blood-health/cryoglobulinemia

  Cryoglobulinemia is characterized by the abnormal precipitation of cryoglobulins in the patients serum leading to end-organ system failure. […] What Are the Diagnostic Tests for Cryoglobulinemia? […] Physical Examination: The healthcare provider should clinically examine the patient thoroughly when diagnosing this disease. Care should be taken if clinical symptoms like purpura, joint pain, erythematous rashes, skin ulcers, and muscle pain are associated with an underlying hematologic disorder. […] Cryoglobulin Test: Measurement of cryoglobulin to show the presence of cryoglobulins should be done essentially. This is known as the cryoglobulin test, which is a laboratory procedure that includes several steps. To perform the cryoglobulin test, the technician usually collects approximately 20 mm (milliliters) of blood and maintains it at 37 degrees Celsius. Anticoagulants are not added to the blood. It is then followed by the centrifugation and refrigeration of the serum to allow coagulation of the abnormal cryoglobulin.

• #3 RheumaKnowledgy » Cryoglobulinemia

  https://www.rheumaknowledgy.com/cyroglobulinemia/

  Diagnostic Tests: Serum collection, processing, and characterization of cryoglobulins must be carried out using proper procedure. Complement levels should be measured. Some cryoglobulins may demonstrate rheumatoid factor activity and should be tested for it. Hepatic enzyme elevations may be related to liver involvement or underlying hepatitis C or B infection, which should be assayed. Peripheral neuropathy or mononeuritis can be confirmed by nerve conduction tests. In some cases, biopsy of a nerve that is abnormal on EMG testing is useful. Skin biopsy evidence of leukocytoclastic vasculitis supports the diagnosis of cryoglobulinemia. […] Keys to Diagnosis: Palpable purpura in a dependent distribution is most suggestive of cryoglobulinemia. A systemic disorder with involvement of the skin, kidney, peripheral nerves, and liver should suggest the possibility of cryoglobulinemia.

• #3 Cryoglobulinemic Vasculitis – Vasculitis Foundation

  https://vasculitisfoundation.org/education/vasculitis-types/cryoglobulinemic-vasculitis/

  Nerve conduction and needle tests (electroneuromyography EMG) of the arms and legs, for evaluation of neuropathy […] Biopsy: surgical removal and examination of tissue from an affected blood vessel or organ; bone marrow, skin, liver or kidney biopsy may be ordered, depending on the organ involvement and any co-existing diseases.

• #3 Relevance and diagnostics of cryoproteins

  https://www.degruyter.com/document/doi/10.1515/labmed-2015-0048/html?lang=en

  Cryoproteins still remain largely undiagnosed despite their clinical relevance. Cryoglobulins, cryofibrinogen and cold agglutinins respectively show a significant association with autoimmune diseases and non-Hodgkins lymphoma. […] The analytical part is relatively straightforward; however, the preanalytical phase is a challenge. […] An understanding of the medical and laboratory practice is important in order for those involved in taking blood samples and laboratory testing to cooperate with each other to prevent an early cryoprecipitation, render the antibodies visible and thus to draw the proper diagnostic conclusions. […] The organizational effort is justified by the diagnostic value. […] The reward for such a process is a patients correct diagnosis, whose illness may have been misunderstood for far too long.

• #3 Cryoglobulinemia Workup: Laboratory Studies, Imaging Studies, Procedures

  https://emedicine.medscape.com/article/329255-workup

  To evaluate for serum cryoglobulins, the blood specimen must be collected in warm tubes (37C) in the absence of anticoagulants. The blood sample must be allowed to clot before removal of serum with centrifugation (at 37C). […] Repeat centrifugation is performed to determine cryocrit (volume of precipitate as a percentage of original serum volume). Cryoglobulin concentration may be determined via spectrophotometric analysis. Specific immunologic assays may be used to identify cryoglobulin components (immunoglobulins, light chains, clonality). […] Hepatitis C virus (HCV) testing is required. HCV antibody or HCV RNA testing may be diagnostic. If HCV test results are negative and clinical suspicion remains high, these tests may be performed on the cryoprecipitate. […] Tissue biopsy may be required for diagnosis when patients with vasculitis, renal disease, or both are evaluated.

• #3 CRY_S – Overview: Cryoglobulin, Serum

  https://www.mayocliniclabs.com/test-catalog/overview/80988

  Type I cryoglobulinemia is associated with monoclonal gammopathy of undetermined significance, macroglobulinemia, or multiple myeloma. […] Type II cryoglobulinemia is associated with autoimmune disorders such as vasculitis, glomerulonephritis, systemic lupus erythematosus, rheumatoid arthritis, and Sjogren syndrome. […] Type III cryoglobulinemia usually demonstrates trace levels of cryoprecipitate, may take up to 7 days to appear, and is associated with the same disease spectrum as Type II cryoglobulinemia. […] Negative (positives reported as percent or trace amount) […] If positive after 1 or 7 days, immunotyping of the cryoprecipitate is performed at an additional charge. […] Failure to follow specimen handling instructions may cause false-negative results.

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