Red Cell Aplasia and Chronic Granulocytic Leukaemia

Abstract

Two patients with chronic granulocytic leukaemia developed red cell aplasia during the course of their disease. In one of them, cell culture studies demonstrated the presence in the patient's serum of an IgG inhibitor of haemoglobin synthesis by his own mature erythroblasts and erythroblasts grown in vitro from his erythroid colony forming cells. The IgG fraction was also found to be cytotoxic for the patients' marrow erythroblasts that were present after disappearance of the red cell aplasia. Treatment with corticosteroids resulted in reappearance of the erythroblasts in the marrow and decrease in the transfusion requirement. Red cell aplasia can occur before, at the same time or after the onset of chronic granulocytic leukaemia and may have the same immune pathogenesis as chronic idiopathic pure red cell aplasia. It occurs without busulphan treatment and seems to have no direct relation to the terminal metamorphosis. Treatment of the red cell aplasia with corticosteroids would appear worthwhile as it may reduce the transfusion requirement without affecting the course of the underlying leukaemia.

Pure red cell aplasia as possible early manifestation of chronic myeloid leukemia

“…The pattern of erythroid progenitor cells which was observed in our patient-namely, decreased BM CFU-E and normal BM and highly increased PB BFU-E, does not tit in any of the above categories. This constellation, together with the presence of various immune-diseaserelated symptoms and the normalization of the erythropoiesis during immunosuppressive therapy, may be explained by, and strongly indicates, immunomediated inhibition of mature CFU-E. Two of the previously reported patients with CML and associated PRCA responded to corticosteroids [8]. Two others were refractory to this therapy [9].…”

“…An antibody inhibition of the heme synthesis was demon-strated in one of the patients [8]. Dessypris et a1 [8] have proposed that PRCA in CML may occur during the course of CML as a second disease coexisting at random and that it may be due to the same immune pathomechanisms as other forms of PRCA. They believe that even in such cases, treatment with corticosteroids may favorably influence the course of PRCA without adversely affecting the course of CML.…”

Pure red cell aplasia heralding chronic myelomonocytic leukaemia

“…However, a clonal abnormality may not fully differentiate between PRCA and MDS since cytogenetic abnormalities have been reported in PRCA. Dessypris et al (1981) in a review of 31 patients with PRCA demonstrated an abnormal marrow cell karyotype in one patient. This was the only patient who subsequently developed acute leukaemia.…”

“…The peripheral leucocyte and platelet counts are usually normal (Ammus & Yunis 1987). PRCA has been described in both chronic myeloid (Dessypris et al 1981) and chronic lymphocytic leukaemia (Ammus & Yunis 1987) but only when the disease is already well established. Our patients had PRCA preceding the appearance of CMML.…”

Chronic myeloid leukemia associated with pure red cell aplasia and terminating in promyelocytic transformation

“…Pure red cell aplasia (PRCA) occurs rarely in the course of chronic myeloid leukemia (CML) [ 1-51. Various mechanisms of PRCA in CML have been postulated. Immune-mediated inhibition of erythropoiesis was demonstrated in one case [3] and postulated in two others [2,3]. In the remaining cases, immunosuppressive therapy failed to induce remission of PRCA [ 1,4].…”