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Treatment of dysmyelopoietic/leukemic syndromes with myelofibrosis and megakaryocytic hyperplasia with large doses of cytosine arabinoside.

Eight patients with inaspiratable bone marrows due to significant degrees of fibrosis were treated with high dose cytosine arabinoside because of either documented or suspected coexistent acute leukemia. The peripheral blood counts returned to normal in 7 patients. Remissions were short lived and bone marrow abnormalities persisted suggesting that consolidation therapy should be administered to these patients as soon as their peripheral blood counts are normalized.

Acute Disease↗

A pleural effusion caused by fibrous hematopoietic tumor successfully treated with prednisolone in a patient with agnogenic myeloid metaplasia with myelofibrosis.

A 46-year-old woman suffering from agnogenic myeloid metaplasia (AMM) since 1994 was being followed as an outpatient. In November 1999, she exhibited respiratory failure caused by massive pulmonary effusion, which was speculated to have been produced by chest tumors. A biopsy specimen revealed findings compatible with fibrous hematopoietic tumor (FHT): prominent fibroblasts and fibrosis with scantv megakaryocytes. Serum concentrations of transforming growth factor (TGF)-beta and platelet-derived growth factor (PDGF) were significantly higher than those of other cases of AMM without FHT. The effusion did not respond to administrations of various chemotherapeutic agents, but after prednisolone administration, the effusion disappeared and the tumors also diminished. TGF-beta and PDGF were the possible causes of FHT formation, and for such fibrotic extramedullary regions, the administration of prednisolone should be considered.

Anti-Inflammatory Agents↗

Massive intrahepatic extramedullary hematopoiesis in myelofibrosis.

We describe the sonographic (US) and computed tomographic appearances of a large, solitary tumor in the liver produced by extramedullary hematopoiesis in an 82-year-old patient with agnogenic myeloid metaplasia. Confirmation of this diagnosis was made by US-guided fine-needle aspiration biopsy.

Aged↗

Successful treatment by selective arterial embolization of severe retroperitoneal hemorrhage secondary to bone marrow biopsy in post-polycythemic myelofibrosis.

Severe retroperitoneal hemorrhage represents an infrequent and serious complication of bone marrow biopsy. A 53-year-old man, diagnosed with polycythemia vera 12 years earlier, was submitted to a bone marrow biopsy due to the appearance of anemia with clinical and hematological features suggesting myelofibrotic transformation, a diagnosis that was confirmed by the marrow study. At 2 h of a right anterior iliac bone marrow trephine biopsy, the patient suddenly developed severe pain in the area of the biopsy, with antialgic flexion of the right leg. Computed tomographic (CT) scan of the abdomen showed a 5 x 9.5 cm hematoma in the right iliac and psoas muscles. The patient was initially managed with analgesics and transfusional support, but the pain persisted and a continuous fall in the hematocrit was observed in the following days. Angiographic examination of the right external iliac artery showed contrast extravasation arising from the circumflex iliac branch, which was embolized using polivinyl alcohol particles and one coil. Following such procedure, the patient recovered uneventfully and was discharged in good condition a few days later. This case illustrates the effectiveness of an endovascular approach in providing a fast and minimally invasive treatment for this life-threatening complication of bone marrow trephine biopsy.

Adult↗