Myelofibrosis Associated with Tuberculosis

M YELOFIBROSIS is a disease in which the normal blood-forming elements of the bone marrow are replaced by fibrous tissue with compensatory extramedullary hemopoiesis arising in other organs of the reticulo-endothelial system. Clinically, it is characterized by pains in the long bones, back or abdomen, progressive weakness, pallor and subsequent loss ofweight. The spleen, liver and sometimes the lymph nodes become enlarged and a refractory anemia of the myelophthisic type develops. Immature leukocytes with or without an increase in total count and frequently the platelet count is either decreased or increased. Bone marrow studies show hypoplasia, usually an increase in the megakaryocytes and eventually, fibrosis. The onset of the disease is insidious and it may last from a few months to years, depending upon the degree of compensation, but the eventual outcome is fatal. Myelofibrosis was first described by Heuck’ in 1879, and since that time approximately 100 cases have been reported in the literature under a great variety of titles; the most common of these are ‘ ‘leuco-erythroblastic ‘2 ‘ ‘chronic nonleukemic y’3 and ‘ ‘myelofibrosis associated with a leukemoid blood pieture. ‘ The name ‘ ‘myelofibrosis’ ‘ was first applied to this disease in 1937 by Mettier and Rusk.4 In 1944, Erf and Herbut’ contributed materially to this subject by their extensive review of the literature and classification of myelofibrosis as either a primary disease or a disease secondary to such conditions as benzene or fluorine poisoning, irradiation and malignant extension. No mention is made of the possible etiologic role of tuberculosis, although such a relationship had been previously suggested. Among 91 cases of myelofibrosis reported in the literature, there were 7 cases with definite evidence of active tuberculosis. A summary of the findings in the 7 cases is recorded in table i. The first American to report a case of myelo.fibrosis was Donhauser6 (case A) in 1908. His case was found to have an active tuberculosis involving the mesenteric lymph nodes and he proposed a toxic etiology for the primary marrow disease. One of the cases reported by Dyke7 (case B) in I9 had miliary tuberculosis; the remaining 4 had other bacterial diseases with bone marrow involvement, and he suggested a disseminated bacteremia as an etiologic factor in this disease. Krasso and Nothnagel5 (case C) in 192.5 found atypical tuberculous lesions in their case which they believed were caused by avian tubercu-