Acquired Pure Red Cell Agenesis

Schmid, J.; Kiely, Joseph M.; Pease, Gertrude L.; Hargraves, Malcolm M.

doi:10.1159/000208132

Cited by 66 publications

(7 citation statements)

References 12 publications

(12 reference statements)

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“…In a few cases the anemia first occurred after the thymoma was resected [8,110,111] and in others anemia developed several years after a mediastinal mass was discovered [112,113]. Of these thymomas, 70% have been of spindle cell type and are rarely malignant [8,58,109,110].…”

Section: Secondary Prcamentioning

confidence: 99%

Acquired pure red cell aplasia

Ammus

Yunis

1987

American J Hematol

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Section: Secondary Prcamentioning

confidence: 99%

Acquired pure red cell aplasia

Ammus

Yunis

1987

American J Hematol

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“…Clinically also the red cell aplasia seemed to run a course independent of the course of CGL, as if the two diseases co-existed a t random. Moreover, the development of red cell aplasia before (Schmid et al, 1963), at the same time, or after the diagnosis of CGL seems to indicate that it may have little relation to the CGL. In our patients the red cell aplasia was a transient phenomenon and in the first of them erythroblasts were increased in the marrow at the time the diagnosis of blastic transformation into acute myelomonocytic leukaemia was made.…”

Section: Discussionmentioning

confidence: 99%

Red Cell Aplasia and Chronic Granulocytic Leukaemia

Dessypris¹,

McKee²,

Metzantonakis³

et al. 1981

Br J Haematol

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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.

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“…Standard androgen therapy with oxymetholone, fluoxymesterone, and testosterone have resulted in remission rates of less than 5 % in refractory cases [3] and approximately 12% in initially diagnosed patients [14]. In addition, in one patient a sequential trial of different androgens appeared to result in a response [ 151.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of danazol in pure red cell aplasia

et al. 1986

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Twenty-one unselected patients with refractory chronic anemias of various etiologies were treated with danazol, a synthetic attenuated androgen. All had previously failed treatment with hematinics, androgens, corticosteroids, high-dose intravenous immunoglobulin, antithymocyte globulin, cytotoxic immunosuppressive agents, and/or plasmapheresis. Three patients with pure red cell aplasia and one with aplastic anemia responded. No responses were observed in 11 patients with myelodysplastic syndromes, two patients with myelofibrosis, and two with paroxysmal nocturnal hemoglobinuria. Remission in pure red cell aplasia was maintained with danazol alone in one patient and required combined low-dose prednisone in two. Objective responses occurred in 2 to 3 weeks and therapy generally was well tolerated. To date, one patient with pure red cell aplasia remains in complete remission at 9 months on low-dose danazol alone. We conclude that danazol alone or combined with prednisone may induce and maintain remission in severe refractory pure red cell aplasia and possibly other chronic cytopenias characterized by immunologic marrow suppression. Further trials of danazol in treating these disorders are indicated.

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Acquired Pure Red Cell Agenesis

Cited by 66 publications

References 12 publications

Acquired pure red cell aplasia

Acquired pure red cell aplasia

Red Cell Aplasia and Chronic Granulocytic Leukaemia

Efficacy of danazol in pure red cell aplasia

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