Myeloproliferative Disorders Terminating in Acute Micromegakaryoblastic Leukaemia

Abstract

Two cases of myeloproliferative disorder--one of myelofibrosis with agnogenic myeloid metaplasia and one of chronic granulocytic leukaemia terminating in acute micromegakaryoblastic leukaemia--are presented. The clinical course is described, and results are reported of morphological, cytometric, cytochemical and cytogenetic studies, as well as cell culture of blood cells in soft agar and in fluid medium.

Leukemia with Megakaryocytic Differentiation following Essential Thrombocythemia and Myelofibrosis

“…Very few small series were reported and those are the best studies available to classify and understand these syndromes [34,[48][49][50][51], Reilly et al [10], in a recent publication, claim to present the second case of 'micromegakaryoblastic leuke mia' following Efrati ct al. [9], without reference to those reported by Ottolander et al [4], Egner et al [16], Bearman et al [5], Truong et al [34], and others. Those cases are similar in many aspects to the one presented in this report.…”

“…In 1985 the French American British (FAB) Cooperative Group published their criteria for diagnosis of megakaryoblastic leukemias or acute my eloid leukemia (AML) M7 [6], Based on these criteria, dc novo AML-M7 is extremely rare in the adult population, and usually associated with blastic transformation of chronic myeloid leukemia (CML) [7,8], Micromegakaryocytic leukemias, on the other hand, are reported in the terminal phase of essential thrombocythemia and myelofibrosis with myeloid metaplasia, as well as associated with other hematological malignancies such as Hodgkin's disease and polycythemia vera [9,10]. Based on morphological criteria micromegakaryocytic leukemia differs from AML-M7 by maturation features, circulating megakaryocytic fragments and thrombocythemia [6,[11][12][13][14].…”

Cytogenetic Studies in Megakaryoblastic Leukaemia

“…Only few cases have been reported and no specific chromo somal changes or markers have been described in these patients [1][2][3][4]. We report the chromosomal find ings in 2 patients with micromegakaryoblastic leu kaemia and compare them with the known findings in myelofibrosis, CML, and megakaryoblastic leu kaemia.…”

“…To the Editor, Acute megakaryoblastic leukaemia is a rare form of leukaemia usually complicating myelofibrosis or chronic myeloid leukaemia (CML) [1,2]. Only few cases have been reported and no specific chromo somal changes or markers have been described in these patients [1][2][3][4].…”

“…Their data is al so consistent with other results from our laboratory [1] showing no significant correlation between the platelet aggregate ratio and the serum total cholester ol concentration of 52 men. The plasma concentra tion of nonesterified fatty acids, which may cause or enhance platelet aggregation under some conditions [2] , is usually elevated when diabetes mellitus is poor ly controlled. Since neither we nor Dettori and col leagues dealt with this aspect of lipid metabolism, it remains to be determined whether elevated concen trations of nonesterified fatty acids may be responsi ble for the low platelet aggregate ratios found in 'juvenile-onset' diabetics and poorly controlled 'adult-onset' diabetics.…”