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Pure Red Blood Cell Aplasia

By

Evan M. Braunstein

, MD, PhD, Johns Hopkins University School of Medicine

Last full review/revision Sep 2021
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Acquired pure red blood cell aplasia is a disorder of erythroid precursors that results in an isolated normocytic anemia. White blood cells and platelets are not affected. Symptoms result from anemia and include fatigue, lethargy, decreased exercise tolerance, and pallor. Diagnosis requires demonstration of peripheral normocytic anemia and a normocellular bone marrow biopsy with absence of erythroid precursors. Treatment usually involves treatment of underlying cause and in some cases thymectomy or immunosuppression.

Etiology of Pure Red Blood Cell Aplasia

Pure red blood cell (RBC) aplasia is most often due to an inappropriate immune response causing suppression of erythropoiesis. Well-known causes include

Symptoms of Pure Red Blood Cell Aplasia

Symptoms of pure RBC aplasia are generally mild and relate to the degree of the anemia or to the underlying disorder. The onset of pure red blood cell anemia usually is insidious, often occurring over weeks or months. Symptoms related to anemia include fatigue, lethargy, decreased exercise tolerance, and pallor.

Diagnosis of Pure Red Blood Cell Aplasia

  • Complete blood count (CBC), reticulocyte count

  • Bone marrow examination

Pure RBC aplasia manifests with a normocytic anemia but normal white blood cell and platelet counts. Reticulocytes are decreased.

The bone marrow reveals normal cellularity with a maturation arrest at the proerythroblast stage. In parvovirus B19 infection, giant pronormoblasts may be present. If possible, a marrow burst forming units-erythroid (BFU-E) assay should be obtained to differentiate autoimmune causes of pure red cell aplasia from primary bone marrow disorders such as myelodysplastic syndrome Myelodysplastic Syndrome (MDS) The myelodysplastic syndrome (MDS) is group of disorders typified by peripheral cytopenia, dysplastic hematopoietic progenitors, a hypercellular or hypocellular bone marrow, and a high risk... read more (1 Diagnosis reference Acquired pure red blood cell aplasia is a disorder of erythroid precursors that results in an isolated normocytic anemia. White blood cells and platelets are not affected. Symptoms result from... read more ).

Chest CT is indicated to evaluate for thymoma in the absence of another identified cause.

Diagnosis reference

Treatment of Pure Red Blood Cell Aplasia

  • Immunosuppression

  • Sometimes intravenous immunoglobulin (IVIG) or thymectomy

Pure RBC aplasia has been successfully managed with immunosuppressants (eg, prednisone, cyclosporine, or cyclophosphamide), especially when an autoimmune mechanism is suspected

Pure RBC aplasia secondary to parvovirus infection is treated with intravenous immune globulin.

Thymectomy is done in patients with thymoma-associated pure RBC aplasia; most patients improve but are not always cured.

Key Points

  • Pure red cell aplasia involves isolated erythroid hypoplasia.

  • Immune-mediated suppression of the erythroid cell line is the most likely cause.

  • Bone marrow cellularity is normal with an arrest of erythroid maturation causing a normocytic anemia.

  • Direct treatment of underlying cause with thymectomy, IV immune globulin (IVIG), or immunosuppression.

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