Immune-mediated cytopenia following bone marrow transplantation. Case reports and review of the literature.
We describe 3 cases of immune-mediated cytopenia occurring after bone marrow transplantation (BMT). In 1 case, only the platelet line was affected, whereas in the other 2 cases more than 1 cell lineage was involved simultaneously. Two of the cases presented with falling peripheral blood counts following apparently normal early engraftment, while in 1 of the cases the affected lineage failed to appear in the peripheral blood despite normal engraftment of the other lineages. In all 3 cases the cytopenia improved following the initiation of treatment with systemic corticosteroids. Immune-mediated cytopenia following bone marrow transplantation may occur via alloimmune or autoimmune mechanisms. Alloimmune cytopenias have arisen in the context of major or minor mismatches in the ABO system, but cases related to mismatches in the Rh system and other erythroid and non-erythroid alloantigen systems may also occur. Alloimmune cytopenias have been reported primarily in the setting of allogeneic BMT, whereas autoimmune cytopenias have been reported following both allogeneic and autologous BMT. Immune-mediated cytopenia may present as early as the day of transplant, or as late as many months afterward. The possibility of immune-mediated cytopenia should always be considered when unexpected peripheral blood cytopenia is present, or when unexpected hemolysis develops, following bone marrow transplantation. The diagnosis is supported by a normal appearance of the affected lineage or lineages in the bone marrow, the absence of other apparent causes for the cytopenia, and the presence of the relevant auto- or allo-antibodies in the serum. However, any of these features may be absent in individual cases. The importance of these syndromes lies in the fact that they may be life-threatening, yet they often respond well to steroids or other standard immunosuppressive measures. It is important to be aware that effective prophylactic measures are available for patients receiving ABO- or Rh-incompatible marrow.
Division of Tumor Immunology, Dana-Farber Cancer Institute, Boston, Massachusetts.
T R
TR
Klumpp
C C
CC
Block
M A
MA
Caligiuri
S N
SN
Rabinowe
R J
RJ
Soiffer
J
J
Ritz
D000328:Adult; D000743:Anemia, Hemolytic / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D015139:Blotting, Southern; D016026:Bone Marrow Transplantation / Q000009:adverse effects* / Q000276:immunology; D002874:Chromosome Mapping; D003937:Diagnosis, Differential; D005260:Female; D005434:Flow Cytometry; D005455:Fluorescent Antibody Technique; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use; D007970:Leukopenia / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012150:Polymorphism, Restriction Fragment Length; D011241:Prednisone / Q000008:administration & dosage / Q000627:therapeutic use; D013156:Splenectomy; D013921:Thrombocytopenia / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy
D016756:Immunoglobulins, Intravenous; D011241:Prednisone