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Acute megakaryoblastic leukemia with massive myelofibrosis: complete remission and reversal of marrow fibrosis with allogeneic bone marrow transplantation as the only treatment.

We report results of allogeneic bone marrow transplantation in an 8-year-old boy with acute megakaryoblastic leukemia characterized by intense fibrosis together with 20% blast cells in the bone marrow, who was transplanted without preceding chemotherapy for remission induction. Conditioning comprised cytosine arabinoside, cyclophosphamide and total body irradiation. The donor was his HLA-identical sister. The patient is well with minor chronic graft-versus-host disease and normal hematologic values 670 days post-transplant.

Bone Marrow↗

[Cutaneous extramedullary hematopoiesis in idiopathic myelofibrosis. Description of a case].

The Authors present a case of cutaneous extramedullary hematopoiesis in a 63-year-old male patient with agnogenic myeloid metaplasia. The skin lesions appeared before splenectomy as multiple papules and nodules on the trunk. Histologic examination of a lesion showed all the tree components of the hematopoietic tissue, i.e. myeloid, erythroid and megakaryocytic series. A review of the literature revealed 18 previously reported cases, with polymorphous clinical manifestations of skin involvement. The possibility of extramedullary hematopoiesis should be considered in a patient with agnogenic myeloid metaplasia who shows cutaneous eruptions.

Hematopoiesis↗

[Coma, hyperviscosity syndrome, hyperammonemia and myelofibrosis in a patient with IgG, lambda type multiple myeloma].

A 63-year-old man was admitted to our hospital with tremor and somnolence, followed soon by coma. Anemia and retinal bleeding were observed. The blood smear exhibited rouleaux formation and leukoerythroblastosis. A bone marrow aspiration resulted in dry tap. The biopsy specimens revealed remarkable infiltration of myeloma cells with fibrosis. The M-component of IgG-lambda type and hyper-ammonemia were detected in the serum. Liver and renal functions, however, were within normal range. His consciousness recovered after plasmapheresis. Two courses of VMCP (vincristine, melphalan, carboquone and prednisolone) did not affect the paraproteinemia. Five courses of VAD (vincristine, adriamycin and dexamethasone) could lower the level of IgG. He died of pneumonia. The plasma of some patients with multiple myeloma may contain unidentified factors which increase the plasma ammonia.

Ammonia↗

Acute surgical abdomen and myelofibrosis.

Extramedullary hematopoiesis associated with fibrosis is found frequently in the liver and spleen, but seldom in other organs. Acute abdomen due to extramedullary hematopoiesis has been reported in two patients with intestinal obstruction because of heavy infiltration of the terminal ileum. This report describes the case of a 71-year-old woman with myeloid metaplasia involving the gallbladder mimicking acute cholecystitis. As far as we know, involvement of the gallbladder by extramedullary hematopoiesis has never been reported before.

Abdomen, Acute↗