Hodgkin disease and red cell aplasia

Abstract

A teenage boy with mixed-cellularity Hodgkin disease presented with severe anemia secondary to pure red cell aplasia of marrow without evidence of lymphomatous infiltration or hemolysis. In vitro studies of the patient's serum demonstrated an inhibitor of erythropoietin activity which appeared to be an IgG but which did not directly bind erythropoietin. The patient's anemia resolved and the inhibitor disappeared following chemotherapy for Hodgkin disease. Presumably, the inhibitor was directed at a very early stage of red blood cell production. This phenomenon may be related to other autoimmune manifestations occasionally seen in patients with lymphomas. The case is presented to bring attention to the unusual occurrence of red cell aplasia in Hodgkin disease. Several hypotheses concerning significance and etiology of the anemia are detailed.

Acquired pure red cell aplasia associated with malignant lymphomas: A nationwide cohort study in Japan for the PRCA Collaborative Study Group

“…Hauswirth et al have analyzed more than 100 reported cases in the literatures of non-CLL non-Hodgkin's lymphoma associated with autoimmune hemolytic anemia (AIHA) or Evans' syndrome, and they have reported that warm antibody mediated AIHA was more frequent in B-cell lymphomas, while cold antibody mediated AIHA predominantly occurred in T-cell lymphomas [34]. Some reports demonstrated that serum immunoglobulin showed an inhibitory activity against erythropoiesis in vitro [7,9,21]. Theoretically, the pathogenesis of PRCA in lymphoma patients can PROVECIP, procarbazine, vinblastine and cyclophosphamide, prednisone; R-FND, rituximab, fludarabine, mitoxantrone and dexamethasone; immunoab, immunoabsorbance; CsA, cyclosporine A; EPO, erythropoietin; IVIG, intravenous immunoglobulin.…”

“…We found twenty-two reported cases of lymphoma-associated with PRCA and these can be classified into two groups based on the chronological sequence in the onset of lymphoma and anemia. Twelve patients presented with lymphoma and PRCA simultaneously (Table IV) [7][8][9][10][11][12][13][14][15][16][17][18], 10 patients developed PRCA following lymphoma (Table V) [5,[19][20][21][22][23][24][25][26][27]. Histologic subtypes of lymphoma varied.…”

Bone biopsy in haematological disorders.

“…The balance has swung in favour of a derivation from the mononuclear phagocyte system, though this is by no means universally accepted. [200][201][202] Suspect biopsies were characterised by small granulomatous foci in the endosteal regions, consisting of lymphocytes, plasma cells, histiocytes and mast cells within a network of reticular fibres and capillaries. The incidence of these sarcoid-like granulomatous foci was 2%.…”

Circulating Mononuclear Cells from Pure Red Cell Aplasia of Chronic Lymphocytic Leukemia Suppress in vitro Erythropoiesis

“…IgG Fc-receptorpositive T cells were found to be markedly increased in CLL-PRCA and different studies indicated that these cells induce the erythroid progenitor growth im pairment [17,5,[7][8][9]18]. However, a similar inhibi tion was not induced by the same phenotypic T cells from normal individuals [4][5][6][7][8].…”