Course of Polycythemia

Polycythemia vera: myths, mechanisms, and management

“…Since the criteria employed by Schwartz and Ehrlich for the diagnosis of polycythemia vera and the diagnosis of acute leukemia were stringent, it is ironic that in the one case of polycythemia vera that they accepted as spontaneously developing acute leukemia, the diagnosis was not histologically confirmed. 146 They also emphasized the association of the newly introduced 32 P therapy with acute leukemia in polycythemia vera patients and correctly predicted that this complication would increase in frequency. By contrast, the advocates of 32 P therapy thought that the development of acute leukemia was an inevitable consequence of extended patient survival.…”

“…Indeed, the basis for this concept of the natural history of polycythemia vera was a 1938 publication by Rosenthal and Bassen describing 13 patients from a series of 75 who were thought to be representative of the various hematologic manifestations of the disease. 146 Of the 13 patients. 2 were anemic and thereby thought to manifest the so-called "spent" phase of the disorder, a concept first proposed by Minot and Buckman.…”

“…Initially and still employed to denote the progression of polycythemia vera to a state of bone marrow failure and possibly a prelude to leukemia 145,146 it has also been used to describe a phase of polycythemia vera with splenomegaly without either myelofibrosis or myeloid metaplasia in which erythrocytosis continues unabated with its extent masked by plasma volume expansion. 136 As indicated above, the former use of the term has never been authenticated in a scientifically acceptable fashion and the latter use, of course, is actually contradictory since in the type of patients described, bone marrow function was actually robust.…”

“…It is difficult to understand how this myth attained credibility since there were both anecdotal reports 185,234,235 and observational series 146,147,149,236 that firmly established the prolonged survival of polycythemia vera patients in an era when the mainstays of therapy were phlebotomy, phenylhydrazine, and sporadic irradiation. Unfortunately, these observations were ignored in favor of the first version of the myth which arose from the assumption that leukemia was an inevitable feature of polycythemia vera.…”

Lymphomas and leukemias

“…This syndrome may also appear in a late stage of polycythemia vera. 28 The fact that plasma cell leukemia occurs makes one consider that multiple myeloma, in which the bone marrow is similarly involved, must also be classified as an aleukemic leukemia. In some cases, multiple myeloma may affect the spleen and the lymph nodes without apparent blood changes.…”