Bone marrow transplantation in a child with hemophagocytic lymphohistiocytosis using a less toxic conditioning regimen.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare non-neoplastic, frequently fatal disease of childhood. HLA-matched bone marrow transplantation (BMT) can bring about long-term remission and an eventual cure. METHODS: We report on the beneficial effect of BMT in a 2-month-old male using a less intensive conditioning regimen. The regimen included busulfan at 4 mg/kg/day (total dose 16 mg/kg), etoposide at 300 mg/m2/day (total dose 900 mg/m2), and cyclophosphamide at 50 mg/kg/day (total dose 150 mg/kg). Prophylaxis for graft-vs.-host disease included methotrexate and cyclosporine. RESULTS: An absolute neutrophil count of 500 microL was noticed on + day 12 (engraftment day). At present, i.e., 400 days after the procedure, the patient is asymptomatic, his physical examination is normal, and a slightly increased level of gamma-glutamyl-transpeptidase (GGT) and alkaline phosphatase are the only laboratory abnormalities. CONCLUSIONS: In this case, the conditioning regimen was adequate for the eradication of the disease and allowed persistent engraftment without significant toxicity. The results in our patient suggest that a less toxic regimen is feasible and permits rapid engraftment without compromising the effectiveness of chemotherapy.

[Intermittent interferon alfa in the treatment of hairy cell leukemia]. / Interferón alfa intermitente en el tratamiento de la leucemia de células peludas.

OBJECTIVE: To evaluate if short and intermittent courses of human recombinant interferon alpha (IFN) are useful in the long term palliation of hairy cell leukemia. METHODS: Nine patients with hairy cell leukemia received 3 megaunits of IFN thrice a week for 12 weeks. They received 8 weeks of IFN upon relapse or after 10 months of followup every year. RESULTS: A hematological remission was obtained in all cases before 12 weeks. Only three patients, because of relapse, required therapy before 10 months. All the patients are alive and well after a median followup of 62 months. CONCLUSIONS: Short courses of IFN proved to be an alternative in the treatment of hairy cell leukemia. It is less expensive and was effective in the initial therapy and maintenance of selected patients with this kind of leukemia.

[3 cases of bone marrow necrosis in acute leukemia]. / Tres casos de necrosis de médula ósea en leucemia aguda.

Bone marrow necrosis (BMN) is mostly diagnosed at postmortem examination. It has been observed in association with acute leukemia and other malignant diseases. We report here BMN in two patients with acute myeloid leukemia (AML) and one with acute lymphocytic leukemia (ALL) in whom the diagnosis was made while alive. Two patients died because of intracranial bleeding. One with AML (M5) developed BMN one week after he was treated with a second course of chemotherapy: he had a complete recovery and remains in remission almost five years after the diagnosis. We conclude that antemortem diagnosis of BMN is technically difficult, but as it is not always associated to a fatal prognosis, an early diagnosis and vigorous supportive therapy should be attempted.

Vasculitis in hairy cell leukemia: rapid response to interferon alpha.

A patient with hairy cell leukemia (HCL) and vasculitis was treated only with interferon alpha-2b. A good, rapid response was obtained without using corticosteroids or immunosuppressors. Interferon may prove to be useful for the systemic therapy of patients with vasculitis associated with HCL.