[Plasma cell leukemia: aggressive form of multiple myeloma].
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Biomedical subjects
Publications and source records attributed to N Ifrah.
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The WHO classification describes a group of myelodysplastic/myeloproliferative diseases, including a provisional entity, refractory anaemia with ringed sideroblasts (RARS) associated with marked thrombocytosis, underlining that is a provisional entity without consensus of belonging to myelodysplastic rather than to myeloproliferative syndromes. The authors report two cases with features of refractory anaemia with excess of ringed sideroblasts and marked thrombocytosis. In the first case, RARS is concomitant with thrombocytosis and fits the WHO criteria for this temporary entity. The second case is a typical RARS, who developed a thrombocytosis after several years and emphasizes that a link, at least progressive, exists between RARS and myeloproliferative disorders. The authors summed up the various situations related to secondary or primary acquired sideroblastic anaemia, likewise to primitive and reactive thrombocytosis. The cases of RARS + marked thrombocytosis reported in the literature are few in number and do not allow to settle between a particular form of myelodysplastic syndrome and a myeloproliferative disorder, a fully justified reason to classify these patients in a temporary group. To date, there is no codified therapy for this disorders.
The authors report the development of thrombocytopenia purpura in one patient with seropositive and erosive rheumatoid arthritis treated successfully for 11 months with D-penicillamine. Anti-platelet-bound antibodies were present, but also: anti-erythrocyte antibodies with hemolytic anemia (then defining Evans's syndrome): higher level of antinuclear antibodies; intermittent neutropenia. The responsibility of D-penicillamine is discussed, but thrombocytopenia purpura evolved for itself. Glucocorticoids alone, intravenous immunoglobulin, vincristine did not induced remission, which at least occurred under the association danazol-glucocorticoids, without toxicity, especially on the liver function.
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