Autonomous Erythropoiesis during Erythroblastic Crisis of Chronic Myelocytic Leukemia

Srodes, C. H.; Hyde, Emily; Boggs, Dane R.

doi:10.1172/jci107208

Cited by 30 publications

(3 citation statements)

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“…Erythroblastic and monoblastic transformations are uncommon. Srodes et al (1973) described two patients with typical Ph' positive CGL who underwent erythroblastic transformation with megaloblastic erythropoiesis. Megakaryoblastic transformation is similarly uncommon.…”

Section: Discussionmentioning

confidence: 99%

Megakaryoblastic transformation of chronic granulocytic leukaemia. An electron microscopy and cytochemial study.

Bain¹,

Catovsky²,

O’Brien³

et al. 1977

J Clin Pathol

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SUMMARY Morphological, cytochemical, and ultrastructural electron microscopic (EM) studies were performed on blood and bone-marrow cells of a case of Ph'-positive chronic granulocytic leukaemia in megakaryocytic acute transformation. The entire leukaemic cell population was found to consist of megakaryoblasts and megakaryocytes. Intermediate stages of maturation between blasts and micromegakaryocytes were observed at EM level.Micromegakaryocytes, usually mononuclear or binuclear, have been recognised as a significant component of the bone marrow in acute myeloid leukaemia (AML), in the preleukaemic stage of AML, and in refractory anaemia with an excess of myeloblasts. In the patient reported here virtual replacement of the marrow by micromegakaryocytes was the chief manifestation of the transformed stage of Philadelphia positive chronic granulocytic leukaemia (CGL). This case accents the fact that CGL is a stem-cell defect, rather than a defect only of granulocytic precursors, and is a further illustration of the failure of megakaryocyte polyploidisation in myeloid malignancy. Case reportThe patient (IS) was a 44-year-old schoolteacher who had been in good health until January 1975, when she presented for medical attention because of increasing abdominal girth. She gave a history of two months' lethargy, dyspnoea, and mild ankle swelling. She had suffered occasional night sweats. Her only relevant past history was of phlebitis in the right leg one year previously. On examination she was pale and the speen was enlarged 18 cm below the left costal margin; the liver was just palpable. Haematological findingsThe haemoglobin (Hb) was 6-6 g/dl and the leucocyte count was 290 x 109/l with 1 % blasts, 7% promyelocytes, 17 % myelocytes, 25 % metamyelocytes, 39 % neutrophils, 3 % basophils, and 3 % eosinophils. There was one nucleated red cell per 100 white blood cells. The neutrophil alkaline phosphatase score (NAP) was 2. Direct examination of the peripheral blood revealed the presence of the Philadelphia chromosome (Ph'). A bone marrow aspirate showed the marrow to be markedly hypercellular with hyperplasia of neutrophil, eosinophil, and basophil lines. Megakaryocytes were increased and an increased percentage of mononuclear and non-segmented forms was noted. A diagnosis of chronic granulocytic leukaemia was made.The initial treatment was by leucapheresis and peripheral blood leucocytes were frozen and stored in liquid nitrogen (Lowenthal et al., 1976). Subsequently, control was achieved with demecolcine for two months followed by busulphan. Seven months after presentation she was submitted to elective splenectomy as part of a programme to assess the place of splenectomy in chronic granulocytic leukaemia. Before splenectomy the patient was clinically well, and the liver and spleen were impalpable. The Hb was 13X3 g/dl, the leucocyte count was 6-4 x 109/l, and the platelet count was 344 x 109/l. Immature granulocytic cells and circulating erythroblasts were not seen in the peripheral blood.After splenectomy the patient...

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Section: Discussionmentioning

confidence: 99%

Megakaryoblastic transformation of chronic granulocytic leukaemia. An electron microscopy and cytochemial study.

Bain¹,

Catovsky²,

O’Brien³

et al. 1977

J Clin Pathol

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“…While the cytological typing of the acute leukaemia that develops in chronic lymphatic leukaemia may present a problem (Zarrabi, Rosner and Grunwald, 1979), in this case the bone marrow morphology was entirely distinct. While erythroblastic transformation is well recognized in myeloproliferative disorders (Rosenthal, Canellos and Grabruck, 1977;Scott, Ellison and Ley, 1964;Srodes, Hyde and Boggs, 1973;Bank, Larsen and Anderson, 1966;Dammert and Kaipainen, 1960), erythroleukaemia occurring in lymphoproliferations is rare but has occasionally been reported (Durant and Tassoni, 1967;Forbes, 1972;Cardamone, Kimmere and Marshall, 1974). The incrimination of chlorambucil therapy in the causation of malignancy remains difficult to establish in individual cases but has been demonstrated in certain patient groups (Berk et al, 1981;Griinwald, 1974, 1975;Carey, Holland and Sheene, 1967;Goldhirsch et al, 1980).…”

Section: Discussionmentioning

confidence: 99%

Chronic lymphatic leukaemia terminating as erythroleukaemia

Al-Hilali

Edgcumbe

Joyner

1981

Postgraduate Medical Journal

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“…In human erythroleukemia (DiGuglielmo syndrome), responsiveness to humoral (erythropoietin) control mechanisms is apparently retained (7,8). On the other hand, the rare erythroblastic crisis of chronic myelogenous leukemia may be autonomous with respect to erythropoietin (9). Compelling evidence favors a normally operating erythropoietin generating mechanism in polycythemia Vera (10,11).…”

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