Bone marrow - nonneoplastic
Alterations in cellularity
Pure red cell aplasia

Author: Dragos Luca, M.D. (see Authors page)

Revised: 7 July 2017, last major update September 2013

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Bone marrow [title] pure red cell aplasia [title]

Cite this page: Pure red cell aplasia. PathologyOutlines.com website. http://pathologyoutlines.com/topic/bonemarrowpureredcellaplasia.html. Accessed October 21st, 2017.
Definition / general
  • Any defect resulting in failure of bone marrow to produce adequate erythroid cells (eMedicine: Pure Red Cell Aplasia)
  • Near absence of red blood cell precursors in bone marrow with associated anemia and reticulocytopenia
  • Normal numbers of megakaryocytes and white blood cell precursors
Terminology
  • Called either erythroblastopenia or red cell aplasia (RCA)
  • Sustained isolated RCA is called pure red cell aplasia (PRCA)
Epidemiology
  • Incidence of RCA varies by specific subtype, the rarest is Diamond-Blackfan anemia
  • Pure RCA predominates in adolescents and adults but can affect any age
Pathogenesis of secondary pure red cell aplasia
  • Humoral and cell mediated immunity are likely to be responsible
  • Anti-erythroid antibodies may be detected in some patients
  • Also due to allogeneic bone marrow transplant from ABO incompatible donor (treat with aggressive plasmapheresis)
  • May be due to antibodies to recombinant erythropoietin in those with chronic renal failure
Etiology
  • Primary (idiopathic) or secondary (acquired)
  • Associated with at least 40 different drugs; phenytoin is most common
  • Also associated with thymoma, hematolymphoid neoplasms (T-LGL leukemia most common), carcinoma, infection (parvovirus B19, mumps, hepatitis, EBV, rarely HIV), immune disorders
Clinical presentation and diagnosis
  • Patients present with severe normochromic, normocytic to slightly macrocytic anemia and marked reticulocytopenia
  • Occult parvovirus B19 infection must be excluded by PCR testing in all cases of secondary PRCA
  • Evaluation for any underlying condition known to be associated with PRCA is also necessary
Laboratory
  • Profound normocytic, normochromic anemia with reticulocytopenia and without polychromasia
  • Other blood parameters generally unremarkable
Case reports
Treatment
  • Steroids and immunosuppressants, antilymphocyte globulin, plasmapheresis, IV immunoglobulins
Microscopic (histologic) description
  • Bone marrow examination usually reveals complete loss of erythroid lineage
  • Sometimes scattered, morphologically unremarkable erythroblasts may be seen especially with IHC
  • Other hematopoietic lineages are morphologically normal and show intact maturation
Microscopic (histologic) images

Images hosted on other servers:

Bone marrow aspirate shows no erythroid elements

Bone marrow biopsy with no erythroid colonies

No erythroid precursors