Pierre, Robert V.

Abstract Amyloid fibrils consist in part of immunoglobulin light chains. Because light chains are synthesized by plasma cells, it seems reasonable to treat patients with amyloidosis with alkylating agents. Two patients who had primary amyloidosis and were treated with melphalan subsequently developed a rapidly fatal acute leukemia. Since melphalan may play a significant role in the development of acute leukemia, we suggest that alkylating agents not be used in the treatment of patients with amyloidosis without full consideration of risks involved, particularly until it has been proved that these drugs are beneficial.

Abstract A light and electron microscopic study was made of the circulating platelets from five patients with variants of the chronic myeloproliferative diseases (agnogenic myeloid metaplasia, chronic granulocytic leukemia, and "megakaryocytic leukemia"). Morphologic abnormalities were found in at least some of the platelets in all of the patients. Presence of giant forms, paucity or absence of granulation, disorganization and scarcity of the microtubules, and haphazard distribution and hypertrophy of the dense tubular and open canalicular systems constituted the main abnormalities. Additionally, there were features of platelet immaturity, including abundant rough endoplasmic reticulum and ribosomes and presence of Golgi profiles, centrioles, and nuclear remnants. The relationship, if any, of these morphologic abnormalities to the hemostatic defects in myeloproliferative diseases remains to be elucidated.

Abstract A patient with chronic granulocytic leukemia is presented with documented central nervous system myeloblastic transformation, while in hematologic remission. This transformation was confirmed by the demonstration of myeloblasts with the Philadelphia (Ph1) chromosome in the cerebrospinal fluid. The course of his illness was unusual in that the initial single manifestation of the acute myeloblastic transformation was an acute meningeal leukemia. Intrathecal Methotrexate treatment resulted in a remission for three months, following which general systemic involvement by acute myeloblastic leukemia ocurred.