Bone marrow nonneoplastic
Alterations in cellularity
Pure red cell aplasia
Author: Dragos C. Luca, M.D.
Last author update: 1 September 2013
Last staff update: 22 July 2019
Copyright: 2002-2019, PathologyOutlines.com, Inc.
PubMed Search: Bone marrow [title] pure red cell aplasia [title]
Table of Contents
Definition / general | Terminology | Epidemiology | Pathogenesis of secondary pure red cell aplasia | Etiology | Clinical presentation and diagnosis | Laboratory | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Luca DC. Pure red cell aplasia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bonemarrowpureredcellaplasia.html. Accessed April 26th, 2024.
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
- 19 year old woman with Mycophenolate mofetil treatment for SLE (Lupus 2013;22:213)
- 43 year old man with CD8+ lymphocytosis in bone marrow (J Clin Pathol 2005;58:1118)
- 70 year old woman with associated thymoma and Good syndrome (Ann Thorac Surg 2011;91:1620)
- 73 year old man following erythropoietin treatment (Rinsho Ketsueki 2012;53:110)
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:
No erythroid precursors
