Niedokrwistość aplastyczna
Zapobieganie i profilaktyka

Niedokrwistość aplastyczna wymaga unikania ekspozycji na czynniki toksyczne, takie jak insektycydy, herbicydy, rozpuszczalniki organiczne oraz benzen, który jest szczególnie niebezpieczny. W przypadku identyfikacji leku wywołującego chorobę, pacjent powinien unikać jego ponownego stosowania, a zdarzenia te należy zgłaszać do systemu monitorowania działań niepożądanych. Leczenie wspomagające obejmuje profilaktykę zakażeń u pacjentów z neutropenią (neutrofile <0,2×10^9/L przez >7 dni), w tym stosowanie leków przeciwgrzybiczych i przeciwwirusowych, a także rozważanie profilaktyki przeciwbakteryjnej (np. lewofloksacyna) i przeciw Pneumocystis jirovecii, choć brak jest jednoznacznych wytycznych. Zalecenia profilaktyczne obejmują unikanie kontaktu z chorymi, higienę rąk, szczepienia (np. przeciw grypie), unikanie publicznych basenów oraz bezpieczne praktyki żywieniowe (unikanie surowych pokarmów, serów dojrzewających, niepasteryzowanych produktów). Przetaczanie składników krwi powinno być wykonywane z użyciem zubożonych leukocytarnie i napromieniowanych koncentratów, przy hemoglobinie <6 g/dl oraz płytkach <10×10^9/L (lub <20×10^9/L przy dodatkowych czynnikach ryzyka krwawienia).

Profilaktyka pierwotna niedokrwistości aplastycznej

Dla większości przypadków niedokrwistości aplastycznej nie ma znanych skutecznych metod profilaktyki pierwotnej. Istnieją jednak czynniki środowiskowe, których unikanie może zmniejszyć ryzyko rozwoju tej choroby12. Do najważniejszych działań profilaktycznych w tym zakresie należy ograniczenie ekspozycji na:

  • Insektycydy3
  • Herbicydy1
  • Rozpuszczalniki organiczne4
  • Środki do usuwania farb3
  • Inne substancje toksyczne5

Szczególną ostrożność należy zachować przy kontakcie z benzenem, który jest używany w produkcji farb, tworzyw sztucznych, benzyny silnikowej, insektycydów i leków5. W przypadkach, gdy ekspozycja na takie chemikalia nie może być uniknięta, należy stosować bezpieczne praktyki obchodzenia się z nimi2.

Jeżeli zidentyfikowano lek jako czynnik wywołujący niedokrwistość aplastyczną, pacjent powinien zostać poinformowany o konieczności unikania tego leku w przyszłości. Przypadki leków powiązanych z rozwojem niedokrwistości aplastycznej powinny być zgłaszane w ramach systemu monitorowania działań niepożądanych leków6.

Profilaktyka wtórna i powikłań niedokrwistości aplastycznej

Leczenie wspomagające i profilaktyka powikłań stanowią istotny element postępowania u pacjentów z niedokrwistością aplastyczną. Zabiegi te są niezbędne zarówno przed, w trakcie, jak i po inwazyjnym leczeniu przyczynowym7.

Profilaktyka zakażeń

Ze względu na neutropenię pacjenci z niedokrwistością aplastyczną są bardziej narażeni na zakażenia8. Zalecenia profilaktyczne obejmują:

  • Unikanie kontaktu z osobami chorymi9
  • Unikanie przebywania w dużych skupiskach ludzi10
  • Częste mycie rąk mydłem i czystą, bieżącą wodą przez co najmniej 20 sekund9
  • Regularne szczotkowanie zębów dla zapobiegania infekcjom zębów i dziąseł11
  • Coroczne szczepienie przeciwko grypie oraz stosowanie innych szczepień ochronnych zgodnie z zaleceniami lekarza12
  • Unikanie korzystania z publicznych basenów i jacuzzi, szczególnie jeśli na skórze są otarcia lub zadrapania11

Wszyscy pacjenci z ciężką neutropenią (liczba neutrofili poniżej 0,2×109/L przez ponad tydzień) powinni otrzymać profilaktyczne leki przeciwgrzybicze13. Pacjenci poddani leczeniu immunosupresyjnemu powinni również otrzymywać leki przeciwwirusowe6.

W kwestii profilaktyki przeciwbakteryjnej u pacjentów z ciężką niedokrwistością aplastyczną stosowane są różne praktyki. Niektórzy lekarze stosują rutynową profilaktykę lewofloksacyną14, choć nie ma jednolitych wytycznych dotyczących profilaktyki przeciwbakteryjnej7.

Kontrowersyjne pozostaje stosowanie profilaktyki przeciwko Pneumocystis jirovecii. Standardowe wytyczne nie zalecają rutynowo takiej profilaktyki u pacjentów z niedokrwistością aplastyczną15, jednak około dwóch trzecich specjalistów stosuje rutynową profilaktykę przeciwko temu patogenowi16. Potrzebne są dalsze badania, aby ocenić, czy korzyści ze stosowania chemoprofilaktyki przeciwko Pneumocystis jirovecii przewyższają ryzyko u pacjentów z bardzo ciężką niedokrwistością aplastyczną17.

Przetaczanie składników krwi

Istotnym elementem leczenia wspomagającego jest przetaczanie odpowiednich składników krwi7:

  • Należy stosować zubożone leukocytarnie i napromieniowane koncentraty krwinek czerwonych i płytek krwi7
  • Wskazaniem do przetoczenia koncentratu krwinek czerwonych jest spadek stężenia hemoglobiny poniżej 6 g/dl7
  • Profilaktyczne przetoczenia płytek krwi powinny być wykonywane, gdy liczba płytek spada poniżej 10×109/L7
  • U pacjentów z dodatkowymi czynnikami ryzyka krwawienia, takimi jak gorączka lub posocznica, zaleca się wyższy próg profilaktycznego przetaczania płytek wynoszący 20×109/L6

U pacjentów kwalifikowanych do przeszczepienia krwiotwórczych komórek macierzystych (allo-HSCT) przetoczenia należy stosować wybiórczo, aby zmniejszyć ryzyko immunizacji przeciwko antygenom dawcy7. Zaleca się konsultację ze specjalistą w dziedzinie transfuzjologii medycznej w sprawie przetaczania krwi zgodnej fenotypowo, aby zmniejszyć ryzyko alloimmunizacji13.

Profilaktyka krwawień

Ze względu na małopłytkowość pacjenci z niedokrwistością aplastyczną są narażeni na zwiększone ryzyko krwawień9. Zalecenia profilaktyczne obejmują:

  • Unikanie aktywności, które mogą spowodować uraz18
  • Unikanie sportów kontaktowych i ryzyka upadku19
  • Unikanie aktywności mogących prowadzić do urazu głowy lub brzucha19
  • Konsultacja z lekarzem w sprawie bezpiecznych aktywności fizycznych9

U kobiet miesiączkujących należy rozważyć stosowanie leków hormonalnych w celu uniknięcia obfitych krwawień miesięcznych spowodowanych małopłytkowością18.

Należy unikać leków, które mogą zaburzać funkcję płytek krwi, takich jak aspiryna czy ibuprofen. W razie potrzeby można stosować paracetamol w celu łagodzenia bólu19.

Bezpieczne odżywianie

Ze względu na zwiększone ryzyko zakażeń pokarmowych, pacjenci z niedokrwistością aplastyczną powinni przestrzegać zasad bezpiecznego odżywiania20:

  • Unikanie surowych lub niedogotowanych pokarmów20
  • Unikanie serów dojrzewających i niepasteryzowanego mleka lub soków11
  • Dokładne gotowanie wszystkich pokarmów20
  • Mycie i obieranie świeżych owoców i warzyw przed podaniem20
  • Unikanie jedzenia w barach sałatkowych i restauracyjnych bufetach20

W razie pytań dotyczących diety warto skonsultować się z dietetykiem20.

Profilaktyka GVHD po przeszczepieniu komórek krwiotwórczych

Przeszczepienie krwiotwórczych komórek macierzystych (allo-HSCT) jest jedną z metod leczenia niedokrwistości aplastycznej, jednak wiąże się z ryzykiem wystąpienia choroby przeszczep przeciwko gospodarzowi (GVHD). Stosowane są różne schematy profilaktyki GVHD21:

Cyklofosfamid po przeszczepieniu

Jednym z obiecujących podejść do ułatwienia wszczepienia i zmniejszenia ryzyka GVHD jest stosowanie cyklofosfamid-po-przeszczepieniu/” title=”cyklofosfamid po przeszczepieniu” class=”to-tag” data-termid=”75197″>cyklofosfamidu po przeszczepieniu (PTCy) w profilaktyce GVHD21. Badania wykazały, że GVHD i odrzucenie przeszczepu można zapobiec u pacjentów z ciężką niedokrwistością aplastyczną powyżej 40 roku życia za pomocą cyklofosfamidu po przeszczepieniu22.

Grupa badawcza z Hopkins stosuje globulinę antytymocytową (ATG), fludarabinę, cyklofosfamid i PTCy zawsze z napromieniowaniem 400 cGy zarówno w leczeniu pierwszego rzutu, jak i opornej choroby, a także w przypadku zarówno niezgodnych, jak i zgodnych dawców21.

Ruksolitynib w profilaktyce GVHD

Dodanie ruksolitynibu do standardowego schematu profilaktyki GVHD jest bezpieczną i wysoce skuteczną metodą łagodzenia GVHD z lepszym wynikiem allo-HSCT23. Badania wykazały, że dodanie ruksolitynibu do standardowego schematu profilaktyki GVHD znacząco zmniejsza częstość ostrej GVHD, przyspiesza rekonstytucję immunologiczną i zmniejsza powikłania infekcyjne23.

W badaniu pilotażowym ruksolitynib był podawany na początku leczenia kondycjonującego i kontynuowany do 3 miesięcy po przeszczepieniu w dawce 5 mg dwa razy dziennie23. Dodanie ruksolitynibu znacząco zmniejszyło częstość zakażeń bakteryjnych/grzybiczych23.

Trwają badania kliniczne, które testują, jak dobrze działa schemat profilaktyki GVHD oparty na ruksolitynibie przed, w trakcie i po przeszczepieniu krwiotwórczych komórek macierzystych (HCT) u starszych pacjentów z nabytą niedokrwistością aplastyczną24.

Profilaktyka reaktywacji WZW typu B

U pacjentów z niedokrwistością aplastyczną związaną z zapaleniem wątroby, którzy poddawani są allo-HSCT, zaleca się stosowanie analogów nukleotydów (np. entekawir, dizoproksyl tenofowiru, alafenamid tenofowiru) w zapobieganiu reaktywacji HBV i ewentualnym leczeniu zakażenia HBV po allo-HCT25.

Profilaktyka zakażenia HBV u pacjentów z wysokim ryzykiem reaktywacji HBV jest tania, łatwa i niesie niskie ryzyko działań niepożądanych25.

Profilaktyka nawrotów niedokrwistości aplastycznej

Około 30-40% pacjentów doświadcza nawrotu choroby po odstawieniu cyklosporyny. Wielu osiąga kontrolę choroby po ponownym wprowadzeniu cyklosporyny, ale pozostają bezterminowo zależni od tego leku26.

Badane są nowe metody zapobiegania nawrotom niedokrwistości aplastycznej po odstawieniu cyklosporyny. Jednym z takich podejść jest konwersja z cyklosporyny na sirolimus26.

Stawiana jest hipoteza, że konwersja z cyklosporyny na sirolimus znacząco zmniejszy wskaźnik nawrotów po leczeniu immunosupresyjnym nabytej niedokrwistości aplastycznej26.

Postępowanie w praktyce klinicznej

W przypadku podejrzenia niedokrwistości aplastycznej lub jej niedostatecznego leczenia zaleca się konsultację medyczną. Wczesna diagnoza i leczenie mają kluczowe znaczenie dla zapobiegania potencjalnie nieodwracalnym powikłaniom27.

W przypadku pacjentów z niedokrwistością aplastyczną, szczególnie tych z niedokrwistością aplastyczną typu ciężkiego lub bardzo ciężkiego, wskazane jest współdziałanie z hematologiem (specjalistą od chorób krwi) lub doświadczonym internistą, pediatrą lub lekarzem rodzinnym w celu ustalenia ciężkości i postępowania w zaburzeniu pacjenta przed podjęciem leczenia stomatologicznego po raz pierwszy27.

Odroczenie zabiegu może być wskazane w oczekiwaniu na zgodę medyczną i potencjalne leczenie wszystkich rodzajów niedokrwistości, szczególnie jeśli podejrzewa się, że niedokrwistość jest ciężka i pacjent ma towarzyszące objawy27.

W przypadku przeszczepienia krwiotwórczych komórek macierzystych, planowe zabiegi stomatologiczne, w tym skalowanie i polerowanie, powinny być opóźnione do czasu powrotu układu odpornościowego pacjenta do normy; zwykle zajmuje to 6-12 miesięcy po przeszczepieniu27.

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Aplastic anemia // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/aplastic-anemia
    There’s no known prevention for most cases of aplastic anemia. Avoiding exposure to insecticides, herbicides, organic solvents, paint removers and other toxic chemicals might lower your risk of the disease.
  • #2
    https://www.nccs.com.sg/patient-care/conditions-treatments/-aplastic-anaemia
    There is no effective prevention for most cases of aplastic anaemia. […] Reducing exposure to these substances may help lower this risk. When exposure to such chemical cannot be avoided, safe handling practices should be employed.
  • #3 Aplastic Anemia: Types, Symptoms, Causes & Treatment
    https://www.webmd.com/a-to-z-guides/aplastic-anemia
    Aplastic anemia is a rare condition and there aren’t any recommendations to prevent it. In some cases, it may happen from toxic exposures. You could take steps to avoid contact with chemicals including: […] Insecticides […] Herbicides […] Organic solvents […] Paint removers.
  • #4 Aplastic Anaemia: Sign and Symptoms, Causes, Risk factors, Diagnosis, Complications, Treatment and Prevention – MedicoInfo
    https://medicoinfo.org/aplastic-anaemia-sign-and-symptoms-causes-risk-factors-diagnosis-complications-treatment-and-prevention/
    There’s no prevention for most cases of aplastic anemia. Avoiding exposure to radiation, insecticides, herbicides, organic solvents, paint removers and other toxic chemicals might lower risk of the disease.
  • #5 Aplastic anemia – USZ
    https://www.usz.ch/en/disease/aplastic-anemia/
    In most cases, you cannot prevent aplastic anemia. Good body protection or, if possible, restraint is only recommended when handling toxic substances. Particular care should be taken with benzene, which is used in the production of paints, plastics, motor gasoline, insecticides and medicines. […] The earlier the disease is detected and treated, the better the chances of survival. […] If toxins or medications are the cause of aplastic anemia, contact with these must of course be prevented or an alternative medication chosen. Then the chances are good that the blood values will normalize again after a while.
  • #6 Aplastic Anaemia: Causes and Treatment | Doctor
    https://patient.info/doctor/aplastic-anaemia
    If a drug trigger can be identified, the patient should be advised never to take the drug again. If a drug is linked with the development of aplastic anaemia this should be reported through the Yellow Card Scheme. […] Prophylactic platelet transfusions should be given to stable aplastic anaemia patients receiving active treatment. A threshold pre-transfusion platelet count of 10 x 109/L should be used. In patients judged to have additional risk factors for bleeding, such as fever or sepsis, a higher prophylactic transfusion threshold of 20 x 109/L is recommended. […] Aplastic anaemia patients who are severely neutropenic should be given prophylactic antibiotics and antifungal therapy according to local policies. […] Aplastic anaemia patients receiving immunosuppressive therapy should also receive prophylactic antiviral agents, although routine prophylaxis against Pneumocystis jirovecii is not necessary.
  • #7 Narrative review of aplastic anemia—the importance of supportive treatment – Urbanowicz – Annals of Palliative Medicine
    https://apm.amegroups.org/article/view/58521/html
    Aplastic anemia (AA) is a rare, life-threatening syndrome of bone marrow failure resulted from bone marrow hypoplasia or aplasia, leading to pancytopenia (not only anemia). […] All patients diagnosed with AA require appropriate supportive treatment adapted to the current clinical situation. Supportive treatment is necessary both before, during and after invasive causal treatment, it mainly involves the transfusion of leukocyte-depleted blood components, the use of anti-infectious prophylaxis or treatment of infections. […] Patients after IST and allo-HSCT treatment require long-term symptomatic management including platelet cell concentrate and red blood cell concentrate transfusions and treatment of infectious complications. […] For any patient for allo-HSCT, transfusions should be used selectively to reduce the risk of immunization to donor antigens.
  • #7 Narrative review of aplastic anemia—the importance of supportive treatment – Urbanowicz – Annals of Palliative Medicine
    https://apm.amegroups.org/article/view/58521/html
    The indication for the administration of red blood cell concentrate transfusions is a decrease in hemoglobin (HGB) concentration below 6 g/dL and prophylactic platelet transfusions should be performed when the patients platelet count (PLT) drops below 10109/L. […] There are no specific guidelines for the prophylactic antibacterial or antifungal treatment of patients with VSAA/SAA. […] The benefits and risks of vaccines in AA also remain controversial because of the risk of immune activation, and some AA guidelines do not recommend vaccination except for patients after allo-HSCT. […] Supportive treatment should also include the transfusion of leukocyte-depleted and irradiated blood components, platelet and red blood cell concentrates, necessary in causally treated patients, and in elderly patients with anemia and thrombocytopenia, in whom aggressive treatment is contraindicated.
  • #8 Infections in patients with aplastic Anemia in Chiang Mai University | BMC Hematology | Full Text
    https://bmchematol.biomedcentral.com/articles/10.1186/s12878-018-0129-9
    Infections occurred in 32.8% in patients with AA in Chiang Mai University. Bacterial infections, especially gram-negative bacilli were the major cause of IEs in patients with AA as well as the most common cause of death. Prevention of infection including infectious control policy, early detection and early treatment of infections, and development of antibiotic guideline according to institution-based epidemiology data will lead to effective strategies to against infections in patients with AA. […] The current study confirmed that the important risk factor of infection in patients with severe AA is severe neutropenia. Approximate 80% of IEs in this study occurred in patients with ANC500/mm3. Moreover, severe neutropenia was also associated with severe IEs, shorter duration from diagnosis to IEs, and higher mortality rate with mean ANC of the patients who died during this study was 149/mm3. […] Antimicrobial prophylaxis including co-trimoxazole, acyclovir, fluconazole, and lamivudine were prescribed in some patients who received immunosuppressive therapy according to physicians decision. No other antimicrobial prophylaxis for gram-negative bacteria was used in this study.
  • #9 Aplastic Anemia | University Hospitals
    https://www.uhhospitals.org/health-information/health-and-wellness-library/article/diseases-and-conditions/aplastic-anemia
    Managing aplastic anemia includes working closely with your provider and following your treatment plan. Tell your provider about any symptoms you are having. You are more at risk of infections so you should: […] Stay away from people who are sick. […] Not be around large crowds. […] Wash your hands often. Use soap and clean, running water (warm or cold) and scrub your hands for at least 20 seconds. […] Not eat foods that are not cooked all the way through. […] Brush your teeth regularly. […] Get your annual flu shot and other vaccines as directed by your provider. […] You may be at higher risk of bleeding if you have a low platelet count. Stay away from activities that could cause an injury. Ask your provider what physical activities are safe for you. You may need to stay away from contact sports.
  • #10 Aplastic Anemia | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/a/aplastic-anemia.html
    Aplastic anemia is a serious illness. Treatment often depends on the underlying cause. For certain causes, you may get better after treatment. But the condition can come back. To treat the low blood counts, early treatment may include: […] Preventive antibiotic therapy […] Good hygiene to prevent infection […] Special care when making food, such as only eating well-cooked foods […] Staying away from construction sites, which may be a source of certain fungi […] You are more at risk of infections so you should: […] Stay away from people who are sick […] Not be around large crowds […] Wash your hands often. Use soap and clean, running water (warm or cold) and scrub your hands for at least 20 seconds […] Not eat foods that are not cooked all the way through […] Get your annual flu shot.
  • #11 Content – Health Encyclopedia – University of Rochester Medical Center
    https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=160&contentid=1
    Preventing infection. Make sure your child gets a flu shot every year and ask their provider about other preventive vaccines. Stay away from crowds and sick friends or relatives, especially during cold and flu season. Remind your childand the rest of the familyto wash hands often. Regular dental care will help prevent tooth and gum infections. Keep your child out of public pools and hot tubs, especially if your childs skin has any open cuts or scrapes. Don’t let anyone smoke around your child. This includes in your home and car.
  • #11 Content – Health Encyclopedia – University of Rochester Medical Center
    https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=160&contentid=1
    During and between treatments, its important to prevent complications of aplastic anemia caused by infections, bleeding, or extreme tiredness (fatigue). This is very important when blood cell counts are low. […] Safe nutrition. Its important for your child to eat a healthy diet. Your child may be at risk for foodborne illness. Because of this, they may need to not eat aged cheeses or drink unpasteurized milk or juices. Your child should not eat undercooked or raw foods. Make sure to cook all foods fully. Wash and peel fresh fruits and vegetables before serving. Don’t let your child eat at salad bars or restaurant buffets. If you have dietary questions, ask the health care provider for a referral to a registered dietitian for additional support. […] Safe exercise. Regular exercise and playtime are important, and so is resting between activities. If your child gets short of breath when active, let your provider know. Because your child may be at risk for bleeding, infection, and fatigue, contact sports are usually discouraged. If your child is school-age, work with teachers and coaches so your child can take part safely in peer activities.
  • #12 Living With Aplastic Anemia | Hematology-Oncology Associates of CNY
    https://www.hoacny.com/patient-resources/blood-disorders/aplastic-anemia/living-aplastic-anemia
    Most people who have aplastic anemia may be at higher risk for infections due to low white blood cell counts. Ask your doctor about ways to lower your risk of infection. For example, you may want to: […] Get a yearly flu shot and pneumonia vaccine. Ask your doctor whether these shots will benefit you. […] Know the signs of infection, such as fever. Call your doctor right away if you think you have an infection.
  • #13 Aplastic Anemia – Aplastic Anemias – Anemia and Other Nonmalignant Blood Disorders – Hematology – Diseases – McMaster Textbook of Internal Medicine
    https://empendium.com/mcmtextbook/chapter/B31.II.15.1.7.
    1) Initiate prophylactic antifungal treatment in patients with prolonged neutropenia (0.2109/L for 1 week). Antiviral treatment should be offered to patients receiving immunosuppressive treatment (IST). […] 2) Transfusion support: Leukoreduced packed red blood cells (PRBCs) and platelets should be used only when considered essential (eg, platelet count 109/L or bleeding). Outcomes of HSCT may be compromised by prior transfusion therapy. Seek consultation with a transfusion medicine specialist regarding transfusion of phenotype-matched blood to reduce the risk of alloimmunization.
  • #14 How do you manage prophylactic antimicrobial medications in
    https://www.themednet.org/how-do-you-manage-prophylactic-antimicrobial-medications-in-patients-who-undergo-atg-cyclosporine-eltrombopag-induction-for-severe-aplastic-anemia
    Yes for prophylactic antibacterials after ATG/cyclosporine and eltrombopag treatment of AA. Antiviral with valtrex 500mg oral twice daily and PJP prophylaxis while on immunosuppression with cyclosporine. Antibacterial with levofloxacin and antifungal prophylaxis with posaconazole 300mg oral daily.
  • #15 Epistaxis, Pneumocystis jirovecii pneumonia and aplastic anaemia: chicken or egg? | HKMJ
    https://www.hkmj.org/abstracts/v29n2/158.htm
    Aplastic anaemia is a rare disease in young children that arises from damage to the bone marrow and resident haematopoietic stem cells. […] Pneumocystis jirovecii pneumonia is rarely reported in patients with aplastic anaemia. […] The risk of PJP in patients with aplastic anaemia has not been defined in the literature, but the risk should be low since the T cells are not defective. […] Patients with aplastic anaemia are usually not considered to be at risk of PJP, hence prophylaxis is not routinely prescribed. […] This raises a broader question of whether PJP prophylaxis (eg, co-trimoxazole, dapsone and pentamidine) should be considered in patients with aplastic anaemia, especially those who fulfil the criteria of very severe aplastic anaemia. […] Although standard guidelines do not recommend PJP prophylaxis in patients with aplastic anaemia, further studies should assess whether the benefits of chemoprophylactic agents outweigh the risks in those considered to have very severe aplastic anaemia.
  • #16 North American Pediatric Aplastic Anemia Consortium Symposium | Aplastic Anemia and MDS International Foundation (AAMDSIF)
    https://www.aamds.org/north-american-pediatric-aplastic-anemia-consortium-symposium
    Supportive care, which encompasses blood production transfusion and the prophylaxis and treatment of infections, is an important component in the management of patients with SAA. […] Regarding the prophylaxis of infection during the time of severe neutropenia, 38% report using granulocyte colony stimulating factor (GCSF) in most patients while 69% report using it during an infection if neutropenia has not resolved. […] The use of antibiotic prophylaxis is also variable. One quarter of responders do not use any antimicrobial prophylaxis. Two thirds use routine prophylaxis against Pneumocystis infection and two thirds use routine antifungal prophylaxis, though the specific antimicrobials varied. […] Prospective, multi-institutional studies are necessary to determine how SAA patients are impacted by supportive care practices.
  • #17
    https://journals.lww.com/pccmjournal/fulltext/2021/03001/p0637____1823__pneumocystis_jirovecii_pneumonia.694.aspx
    Aplastic anemia is a rare disease in young children with an often unknown cause. […] The risk of PCP in patients with aplastic anemia has not been defined in the literature, but should be low as the T-cells are not defective. […] Although standard guidelines do not recommend PCP prophylaxis in patients with aplastic anemia, further studies could review whether chemoprophylactic agents for PCP is beneficial to those with very severe aplastic anemia.
  • #18 Aplastic anemia secondary prevention – wikidoc
    https://www.wikidoc.org/index.php/Aplastic_anemia_secondary_prevention
    Effective measures for the secondary prevention of complications of aplastic anemia include avoiding raw meats, dairy products, or fruits. These vegetables or fruits might be colonized by bacteria, fungus, or molds. Salt-limited diet is recommended during therapy with steroids or cyclosporin. These patients should avoid any activity that increases the risk of trauma during periods of thrombocytopenia. Menstruating women should recieve hormonal pills to avoid menstrual cycles that might get heavy due to thrombocytopenia. Patients should be informed of the increased risk of community-acquired infections during periods of neutropenia and lymphopenia, and they should maintain hygiene to reduce the risks of infection. […] Effective measures for the secondary prevention of complications of aplastic anemia include Diet care: Patients with aplastic anemia who have neutropenia or who are receiving immunosuppressive therapy should not consume raw meats, dairy products, or fruits.
  • #18 Aplastic anemia secondary prevention – wikidoc
    https://www.wikidoc.org/index.php/Aplastic_anemia_secondary_prevention
    These patients should avoid any activity that increases the risk of trauma during periods of thrombocytopenia. […] Menstruating women should recieve hormonal pills to avoid menstrual cycles that might get heavy due to thrombocytopenia. […] Patients should be informed of the increased risk of community-acquired infections during periods of neutropenia and lymphopenia. […] Patients should maintain hygiene to reduce the risks of infection.
  • #19 Aplastic Anemia – Together by St. Jude™
    https://together.stjude.org/en-us/conditions/blood-disorders/aplastic-anemia.html
    Take steps to prevent infection. Wash hands often, keep patient areas clean and clutter free, and stay away from people who are sick. Your child should avoid: […] Talk to your doctor before giving your child any medicine. Medicines like aspirin or ibuprofen (Motrin, Advil) can keep platelets from working properly. If needed, give acetaminophen (Tylenol) for headache or pain. Do not give any medication for fever without first contacting your care team. […] Know what activities to avoid. If your child has fewer red blood cells, they may get tired more easily or get out of breath. Be sure your child does not overdo physical activity. When platelet counts are low, your child is at higher risk of bleeding. Your child should avoid contact sports, rough play, and activities that could involve falls or injury to the head or stomach. […] Certain activities can be a health risk if your child has aplastic anemia. Limit tiring activities, avoid activities with a risk of injury, and take steps to prevent infection.
  • #20 Living with Aplastic Anemia
    https://healthlibrary.chnola.org/library/PreventionGuidelines/160,1
    During and between treatments, its important to prevent complications of aplastic anemia caused by infections, bleeding, or extreme tiredness (fatigue). This is very important when blood cell counts are low. […] Here are some basic tips to keep in mind: […] Safe nutrition. Its important for your child to eat a healthy diet. Your child may be at risk for foodborne illness. Because of this, they may need to not eat aged cheeses or drink unpasteurized milk or juices. Your child should not eat undercooked or raw foods. Make sure to cook all foods fully. Wash and peel fresh fruits and vegetables before serving. Don’t let your child eat at salad bars or restaurant buffets. If you have dietary questions, ask the health care provider for a referral to a registered dietitian for additional support.
  • #21
    https://haematologica.org/article/view/haematol.2023.284022
    More recent research efforts in SAA BMT have focused on transplant feasibility using mismatched donors to expand the donor pool. […] The most promising approach to facilitate engraftment and mitigate the risk of GVHD is the use of post-transplant cyclophosphamide (PTCy) for GVHD prophylaxis. […] Given the disease rarity, there have been no comparative studies of conditioning or GVHD prophylaxis, but an important issue remains in 2023 with matched donors, especially in adult patients, where we still need to minimize any complications with BMT, namely cGVHD, mixed chimerism, relapse or clonal evolution, and other transplant-related complications. […] In summary, the Hopkins group uses ATG, fludarabine, cyclophosphamide, and PTCy always with 400 cGy in the upfront and refractory settings as well as with mismatched and matched donors.
  • #22 Post-transplantation cyclophosphamide for GVHD prophylaxis in severe aplastic anemia | Bone Marrow Transplantation
    https://www.nature.com/articles/bmt2010213
    Here, we show that GVHD and rejection can be prevented in SAA patients over the age of 40 with post-transplant CY. […] Given the high risk of GVHD following BMT in patients 40 years, and the high risk of GVHD in patients receiving a BMT from mismatched or unrelated BMT donors, these data suggest that the addition of post-transplantation CY to traditional immunosuppression deserves consideration as GVHD prophylaxis in SAA patients at high risk for GVHD.
  • #23 Addition of ruxolitinib to standard graft-versus-host disease prophylaxis for allogeneic stem cell transplantation in aplastic anemia patients | Bone Marrow Transplantation
    https://www.nature.com/articles/s41409-024-02266-7
    Allogeneic hematopoietic stem cell transplantation (allo-HSCT) offers rapid hematopoietic and immune reconstitution for aplastic anemia (AA). […] Our study sought to investigate the safety and efficacy of the prophylactic use of ruxolitinib in allogeneic HSCT. […] Addition of ruxolitinib to standard GVHD prophylaxis regimen, thus, represents a safe and highly efficient method for the attenuation of GVHD with better outcome of allo-HSCT. […] Therefore, we conducted this pilot study to investigate the efficacy and safety of ruxolitinib in GVHD prophylaxis in AA patients which comprised of predominantly adults with a few pediatric patients. […] Here we show addition of ruxolitinib to a standard GVHD prophylaxis regimen has resulted in a significantly lower incidence of acute GVHD, faster immune reconstitution, and attenuated infectious complications.
  • #23 Addition of ruxolitinib to standard graft-versus-host disease prophylaxis for allogeneic stem cell transplantation in aplastic anemia patients | Bone Marrow Transplantation
    https://www.nature.com/articles/s41409-024-02266-7
    Ruxolitinib was initiated at the start of the conditioning regimen and continued until 3 months post-transplantation with a dose of 5mg twice daily. […] Addition of ruxolitinib significantly reduced the incidence of bacterial/fungal infections in our study. […] Our study is the first to explore the peri-transplant use of ruxolitinib in addition to standard GVHD prophylaxis in AA patients. […] In all, the addition of ruxolitinib to standard GVHD prophylaxis regimen demonstrated high safety and indicated profound efficacy in reducing aGVHD. […] The optimization of PBSC-based transplant protocols is particularly beneficial for recipients who do not have to access to bone marrow graft.
  • #24 Ruxolitinib Based GVHD Prophylaxis Regimen before, during, and after Hematopoietic Cell Transplantation in Older Adult Patients with Acquired Aplastic Anemia – NCIFacebookFollow on XInstagramYoutubeLinkedin
    https://www.cancer.gov/about-cancer/treatment/clinical-trials/search/v?id=NCI-2024-06524
    This phase II trial tests how well a ruxolitinib-based graft versus host disease (GVHD) prevention (prophylaxis) regimen works before, during, and after bone marrow/stem cell transplantation (hematopoietic cell transplantation [HCT]) in patients with acquired aplastic anemia. […] The current study aims to assess whether adding ruxolitinib to a standard GVHD prevention regimen may reduce the risk of Grade II-IV acute and chronic GVHD after bone marrow/stem cell transplantation in older patients with acquired aplastic anemia. […] PRIMARY OBJECTIVE: I. To evaluate whether the addition of ruxolitinib to standard cyclosporine (CSA)/mycophenolate mofetil (MMF) +/- sirolimus GVHD prophylaxis will decrease the rate of day 100 acute grades II-IV GVHD in 20 patients with aplastic anemia underdoing non-myeloablative allogeneic hematopoietic cell transplantation compared with historical controls. […] Trial Type prevention
  • #25 Prophylaxis with tenofovir in hepatitis-associated severe aplastic anemia treated with hematopoietic cell transplantation | Prodisteanu | Acta Haematologica Polonica
    https://journals.viamedica.pl/acta_haematologica_polonica/article/view/96892/76794
    Prophylaxis with tenofovir in hepatitis-associated severe aplastic anemia treated with hematopoietic cell transplantation […] Therefore, it is recommended to use nucleotide analogs [i.e. entecavir, tenofovir disoproxil (TDF), tenofovir alafenamide] in the prevention of HBV reactivation and possible treatment of HBV infection after allo-HCT […] This is why preventing HBV reactivation in allo-HCT patients is critical. Our case shows that TDF at a dose of 245 mg/day was effective in this prophylactic strategy. […] In conclusion, this case highlights the importance of prophylaxis of HBV infection in patients who are at high risk of HBV reactivation. This prophylaxis is cheap and easy and carries a low risk of adverse side effects.
  • #26 Sirolimus (Rapamune ) for Relapse Prevention in People With Severe Aplastic Anemia Responsive to Immunosuppressive Therapy | Clinical Research Trial Listing
    https://www.centerwatch.com/clinical-trials/listings/NCT02979873/sirolimus-rapamune-for-relapse-prevention-in-people-with-severe-aplastic-anemia-responsive-to-immunosuppressive-therapy
    People with severe aplastic anemia (SAA) do not make enough red and white blood cells, and/or platelets. […] The drug sirolimus may help by suppressing the immune system. […] To evaluate and compare the usefulness of sirolimus in preventing aplastic anemia from returning after cyclosporine is stopped, compared with stopping cyclosporine alone. […] About 30% to 40% of patients relapse after discontinuation of cyclosporine. Many achieve disease control after the reinitiation of CSA, but remain CSA dependent indefinitely. […] We hypothesize that CSA to SRL conversion will significantly decrease the relapse rate after immunosuppressive therapy for acquired aplastic anemia. […] This study will investigate the safety and efficacy of SRL for preventing relapse in patients -previously treated with IST who remain on CSA. The primary endpoint is rate of relapse at 2 years following conversion from CSA to SRL, versus stopping CSA.
  • #27 Anemia (includes iron deficiency anemia; aplastic anemia [also known as ‘hypoplastic anemia’; includes Fanconi’s anemia]; vitamin B12 deficiency anemia [which includes pernicious anemia]; folate deficiency anemia; hemolytic anemia [which includes sickle c
    https://cdho.org/factsheets/anemia/
    Is medical consult advised? […] Yes, if previously undiagnosed or inadequately treated anemia is suspected. For instance, early diagnosis and treatment is important to prevent potentially irreversible neurologic signs in vitamin B12 and folate deficiency anemias. Similarly, aplastic anemia can be life-threatening, and prompt diagnosis and treatment are critical. […] Yes, if patient/client has sickle cell anemia, thalassemia, or aplastic anemia in which case liaison with the patient/clients hematologist (blood specialist) or experienced internist, paediatrician, or family physician is advisable regarding the severity and management of the patients disorder before undertaking dental hygiene treatment for the first time. […] Is medical clearance required? […] Yes, if anemia has been diagnosed or is suspected.
  • #27 Anemia (includes iron deficiency anemia; aplastic anemia [also known as ‘hypoplastic anemia’; includes Fanconi’s anemia]; vitamin B12 deficiency anemia [which includes pernicious anemia]; folate deficiency anemia; hemolytic anemia [which includes sickle c
    https://cdho.org/factsheets/anemia/
    Yes, pending medical clearance (and potential treatment) for all anemias. […] Yes, if anemia is suspected to be severe and the patient/client has attendant signs/symptoms. […] In the case of hematopoietic stem cell transplantation, elective oral procedures, including scaling and polishing, should be delayed until the patient/clients immune system returns to normal; typically, this takes 6 to 12 months post-transplantation. […] Aplastic anemia causes manifestations related to the generalized decrease in red and white blood cells as well as platelets. Thus, infection (e.g., candidiasis), spontaneous gingival bleeding, petechiae, and purpuric spots may occur intra- and peri-orally. […] Prognosis depends on the underlying cause of anemia, its severity, and the overall health status of the patient/client. Many forms of anemia are curable or at least treatable. Some inherited anemias such as sickle cell anemia can lead to life-threatening complications. Severe or long-lasting anemia can damage the heart, brain, and other organs.