Causes of acquired pure red cell aplasia (PRCA)

Primary PRCA

Idiopathic (autoimmune)

Initial presentation of MDS

Transient erythroblastopenia of childhood (TEC)*

Secondary PRCA

Drugs

Chlorpropamide

Erythropoietin (EPO, recombinant)

Immune checkpoint inhibitors (monoclonal antibodies targeting CTLA4, PD1, or PDL1)

Mycophenolate mofetil

Phenytoin

Trimethoprim-sulfamethoxazole

Zidovudine

Infections

B19 parvovirus (transient or chronic)

Epstein-Barr virus

HIV

Viral hepatitis

Immune disorders

Autoimmune hemolytic anemia

Systemic lupus erythematosus

Rheumatoid arthritis

ABO-incompatible hematopoietic stem cell transplant

Neoplasms

Lymphoid

CLL
LGL leukemia
Hodgkin lymphoma
Non-Hodgkin lymphoma
Multiple myeloma and other plasma cell disorders

Myeloid

Other

Thymoma (approximately 5% of cases)
Cancers with anemia treated with EPO

Pregnancy

This is a partial list intended to summarize the most common causes of PRCA. Lymphocyte and plasma cell disorders are the most common underlying disorders in secondary PRCA. Additional causes (including additional drugs and viral infections) may have been reported. Refer to UpToDate for the evaluation of these disorders.

PRCA: pure red cell aplasia; MDS: myelodysplastic syndrome; EPO: erythropoietin; CTLA-4: cytotoxic T lymphocyte associated antigen 4; PD1: programmed cell death-1; PD-L1: programmed cell death ligand 1; CLL: chronic lymphocytic leukemia; LGL: large granular lymphocyte; NHL: non-Hodgkin lymphoma; CML: chronic myelogenous leukemia; PMF: primary myelofibrosis.

* TEC is an acute, self-limited PRCA seen only in childhood. It is the most common cause of PRCA in children and typically occurs in previously healthy children. The precise mechanism is unknown. Refer to the UpToDate discussion of PRCA in children for details.

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Causes of acquired pure red cell aplasia (PRCA)