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Suppression for the Suppressed: Aplastic Anemia Without a Sibling Donor Match

Suppression for the Suppressed: Aplastic Anemia Without a Sibling Donor Match. Calvin Thigpen, M.D. Hematology/Oncology Fellow University of Mississippi Medical Center. 17-year-old AA Female. Outside Lab Data. What Could This Be?. Systemic lupus erythematosus. Hairy cell leukemia.

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Suppression for the Suppressed: Aplastic Anemia Without a Sibling Donor Match

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  1. Suppression for the Suppressed: Aplastic Anemia Without a Sibling Donor Match Calvin Thigpen, M.D. Hematology/Oncology Fellow University of Mississippi Medical Center

  2. 17-year-old AA Female

  3. Outside Lab Data

  4. What Could This Be? Systemic lupus erythematosus Hairy cell leukemia B12 deficiency Aleukemic leukemia Paroxysmal nocturnal hemoglobinuria Idiopathic myelofibrosis Aplastic anemia Myelodysplastic syndrome

  5. On Arrival • More history • Had recently taken amoxicillin, Lortab, dexamethasone • No prominent exposure to chemicals • No HIV risk factors or adverse behaviors • No family history of pancytopenia

  6. On Arrival • Exam • Hemodynamically stable • Obese • No mental status alterations • Pale conjunctivae • No active bleeding in mouth • No palpable splenomegaly • No rash

  7. Lab Data • CBC (after outside transfusion) • WBC 3.2, ANC 384 • Hgb 7.3 g/dL • Platelets 93,000 • Corrected retic count 0.3 • LDH 142 • B12 526

  8. Further Investigation

  9. She has a brother! • Sibling HLA typed • Transfusions purposefully limited • Bone marrow aspirate and biopsy

  10. Normal Bone Marrow Patient’s Bone Marrow

  11. Treatment Course

  12. Aplastic Anemia • Normal hematopoietic tissue replaced by fat • 3 to 6 cases per million people per year in U.S. • Acquired or inherited • 1 year mortality near 70% if untreated

  13. Etiology – Acquired AA

  14. Pathogenesis

  15. Prognosis • Two determining factors • Age • Younger patients better candidates for transplant • Younger patients respond better to immunosuppression • Severity of aplasia • The lower the counts, the more susceptible to: • Bleeding • Infection

  16. Treatment • 3 possibilities • Matched sibling stem cell transplant • Immunosuppressive therapy • Horse Anti-Thymocyte Globulin (ATG) • Cyclosporine • Steroids • Matched unrelated stem cell transplant

  17. Complications • Transplant • Failure to engraft • GVHD • Immunosuppression • Prolonged time to response (median 95 days) • Relapse • Late hematologic complications • MDS • PNH

  18. Currently

  19. Lessons • Majority of aplastic anemia is idiopathic. • Prognosis depends on age of patient and severity of aplasia. • 1st line treatment for younger patients is matched sibling stem cell transplant.

  20. Lessons • Next best alternative is immunosuppression with ATG and cyclosporine. • Response to immunosuppression may take 3 to 6 months. • Matched unrelated stem cell transplant is a final option.

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