Splenectomy in leukemia and myelofibrosis. Changes in the erythrocyte values.
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Studies were done to test the neutrophil alkaline phosphatase (NAP) synthesizing capacity of the neutrophils of patients with chronic phase chronic myeloid leukemia (CML) and of patients with stable-phase myeloid metaplasia (MM) and to test the NAP synthesis-inducing capacity of monocytes (Mos) of patients with CML. Suspension cultures of the blood light density (LD) cells, LD cells depleted of T-cells and Mos (LD-T-Mo), and cocultures of LD-T-Mo with Mos were performed. NAP synthesis occurred in a normal fashion in LD cell cultures of five of seven patients with CML and of two patients with MM. The NAP synthesis was very slight or did not occur in cultures of LD-T-Mo cells of all patients with chronic-phase CML and MM. However, addition of allogeneic or autologous Mos to the LD-T-Mo cultures restored the NAP synthesis. These results confirm the previous finding that the low or absent NAP in CML is caused by a relative reduction in the monocyte mass and they further indicate the mechanism to be the same for the low or absent NAP in patients with MM. The results also indicate that the NAP-synthesizing capacity of neutrophils of CML and MM patients and the NAP synthesis-inducing capacity of the Mos of patients with CML are normal.
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A 59-year-old man had well-documented agnogenic myeloid metaplasia (AMM) with pancytopenia. Frequent blood transfusions were required over a 10-month period. Androgen therapy was not beneficial and treatment with interferon resulted in severe thrombocytopenia with no decrease in transfusion requirements. Treatment with clodronate at a daily oral dose of 30 mg/kg resulted in a marked decrease in bone marrow fibrosis, and gradual normalization of blood counts over an 8-month period. He has been transfusion independent for the last 33 months. We support the findings of a previous case report that oral bisphosphonate therapy may be of value in patients with AMM.
A 60-year-old man with a myeloproliferative syndrome and extramedullary hematopoiesis had progressive respiratory and cardiac insufficiency during the previous 18 months, with advancing interstitial pulmonary disease on chest x-ray. During analysis of his respiratory disease, results of a transbronchial biopsy showed interstitial involvement with increased numbers of megakaryocytes and other panhematopoietic staining elements. Results of a bone marrow scan demonstrated diffuse replacement of pulmonary interstitium with bone marrow, as a component of known ongoing extramedullary hematopoiesis.
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