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Pure red cell aplasia

Robert T. Means Jr

Article Figures & Data

Figures

Tables

  • Table 1.

    Classification of pure red cell aplasia

    Congenital PRCA
    Diamond-Blackfan anemia
    Acquired PRCA
    Primary
     Primary autoimmune PRCA (includes transient erythroblastopenia of childhood)
     Primary myelodysplastic PRCA
    Secondary, associated with:
    Autoimmune/collagen vascular disorders
     Systemic lupus erythematosus
     Rheumatoid arthritis
     Inflammatory bowel disease
    Other immunologic mechanisms
     ABO-incompatible stem cell transplantation
     Pyoderma gangrenosum
    Lymphoproliferative disorders
     Chronic lymphocytic leukemia
     LGL leukemia
     Hodgkin disease
     Non-Hodgkin lymphomas
     Angioimmunoblastic lymphadenopathy
     Multiple myeloma
     Waldenstrom macroglobulinemia
     Castleman disease
    Other hematologic malignancies
     Chronic myelogenous leukemia
     Chronic myelomonocytic leukemia
     Myelofibrosis with myeloid metaplasia
     Essential thrombocythemia
     Acute lymphocytic leukemia
    Solid tumors
     Thymoma
     Gastric cancer
     Breast cancer
     Biliary cancer
     Lung cancer
     Thyroid cancer
     Renal cell carcinoma
     Carcinoma of unknown primary site
    Infections
     B19 parvovirus
     Human immunodeficiency virus
     T-cell leukemia-lymphoma virus
     Infectious mononucleosis
     Viral hepatitis (hepatitis A, B, C, and E)
     Cytomegalovirus
    Bacterial infections
     Group C Streptococcus
     Tuberculosis
     Bacterial sepsis
    Drugs and toxins
     rhEpo-induced Epo antibody-associated PRCA
     Other drugs (Table 2)
    Other disorders
     Pregnancy
     Riboflavin deficiency
    • Modified from Lipton et al.44

  • Table 2.

    Selected drugs associated with PRCA based on PubMed review

    AgentMultiple reportsMechanism investigated
    Alemtuzumab
    Allopurinol
    Ampicillin
    Azathioprine
    Carbamazepine
    Cephalothin
    Cladribine
    Chlorpropamide
    Chloroquine
    Clopidogrel
    Dapsone/pyrimethamine
    Diphenylhydantoin
    Recombinant Epo
    Estrogens
    Fenoprofen
    Fludarabine
    Interferon-α
    Isoniazid
    Lamivudine
    Leuprolide
    Linezolid
    Micafungin
    Mycophenolate mofetil
    d-Penicillamine
    Phenylbutazone
    Procainamide
    Ribavirin
    Rifampicin
    Sulfasalazine
    Sulindac
    Tacrolimus
    Trimethoprim/sulfamethoxazole
    Valproic acid
    Zidovudine
    • PubMed search performed May 15, 2016. Search terms used were “red cell aplasia” AND the name of the specific drug.

  • Table 3.

    Response of patients with PRCA to immunosuppression

    Agent usedPatients treated (responders/total)% responding
    Corticosteroids50/12839%
    Cytotoxic agents46/11341%
    Cyclosporine A37/4877%
    Antithymocyte globulin10/1953%
    Multiple agents91/13368%
    • Total patients treated, n = 195 (many patients did not respond to treatment or suffered relapses, so 1 patient may be included in more than one treatment modality); primary PRCA, n = 137; secondary PRCA (n = 58). Modified from Lipton et al.44 Results pooled from 4 series.1,13,26,27