Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation.

Hemophagocytic lymphohistiocytosis (HLH) comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by fever, hepatosplenomegaly, and cytopenia. FHL, an autosomal recessive disease invariably fatal when untreated, is associated with defective triggering of apoptosis and reduced cytotoxic activity, resulting in a widespread accumulation of T lymphocytes and activated macrophages. In 1994 the Histiocyte Society initiated a prospective international collaborative therapeutic study (HLH-94), aiming at improved survival.

A randomized trial of treatment for multisystem Langerhans' cell histiocytosis.

Objective: To compare 2 active agents, vinblastine and etoposide, in the treatment of multisystem Langerhans' cell histiocytosis (LCH) in an international randomized study.

Study Design: One hundred forty-three untreated patients were randomly assigned to receive 24 weeks of vinblastine (6 mg/m(2), given intravenously every week) or etoposide (150 mg/m(2)/d, given intravenously for 3 days every 3 weeks), and a single initial dose of corticosteroids.

Results: Vinblastine and etoposide were equivalent (P > or = .

LCH-I: a randomized trial of etoposide vs. vinblastine in disseminated Langerhans cell histiocytosis. The Histiocyte Society.

An international randomized trial in Langerhans cell histiocytosis (LCH) has been initiated by the Histiocyte Society. This report reviews the rationale, design, and progress of LCH-I, which compares etoposide (VP-16) and vinblastine in the treatment of disseminated LCH. Data on the risk of etoposide-associated (therapy-induced) malignancy, in the setting of histiocytosis, are reviewed.

Histiocytes in familial and infection-induced/idiopathic hemophagocytic syndromes may exhibit phenotypic differences.

Familial hemophagocytic syndrome (FHS) and infection-associated hemophagocytic syndrome (IAHS) usually present with fever, pancytopenia, hepatosplenomegaly, signs of hepatic dysfunction, bleeding diathesis, and neurological manifestations. FHS is almost uniformly fatal, and IAHS is associated with high mortality. The only distinguishing characteristics are lack of family history and association with infection in the latter.

The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).

The available information on treatment of sinus histiocytosis with massive lymphadenopathy (SHML) was reviewed. Treatment is not necessary in most instances, but some patients may require surgery, radiation therapy, and/or chemotherapy because of severe disease manifestations. An ideal treatment has yet to be identified.

Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA.

The brains of eight patient with Langerhans Cell Histiocytosis (LCH) were studied with magnetic Resonance Imaging (MRI). One scan was normal and a spectrum of abnormalities was seen in the others. Five patients had absence of the posterior pituitary bright spot, and four with evidence of pituitary dysfunction had a lesion in the region of the hypothalamus.

Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes.

The serum of children with untreated hemophagocytic syndromes contains elevated levels (23,600 to 75,200 U/mL) of soluble interleukin-2 receptor (SIL2R) that returns toward normal with clinical improvement. These levels are in excess of levels previously reported for benign conditions. They are as high as levels reported for HTLV-1-associated adult T-cell leukemia (HATL) and hairy cell leukemia (HCL) in adults and some children with poor-prognosis non-T, non-B, acute lymphoblastic leukemia (ALL).

Histiocytosis syndromes in children: II. Approach to the clinical and laboratory evaluation of children with Langerhans cell histiocytosis. Clinical Writing Group of the Histiocyte Society.

Langerhans cell histiocytosis (LCH) has presented problems in diagnosis and in treatment. The definitive diagnosis is a pathologic one. To advance thorough and uniform assessment of these patients, the elements of a complete clinical and laboratory evaluation are presented by the Histiocyte Society.

Historical perspectives of Langerhans cell histiocytosis.

This brief review of the history of Langerhans cell histiocytosis brings us into touch with the history of medicine of the last 100 years and the opportunity to reflect on the progress in medicine in general. Hand, Christian, Schüller, Letterer, and Siwe represent far more than names to attach to eponyms. This historical overview takes us from the first descriptions of the disease in the literature through the modern efforts to take the X out of histiocytosis-X.

Circulating anticoagulant to animal plasmas in a cardiac patient.

A circulating anticoagulant against bovine, equine, guinea pig, and sheep plasmas developed in a 15-year-old cardiac patient. He had been exposed to both bovine and porcine heparin over a period of 13 years, and had a porcine valve placed four years before the anticoagulant was noted. There was no anticoagulant activity detected against human, rat, or porcine plasma, and an equivocal reaction against rabbit plasma.

Thrombocytopenic purpura following varicella-zoster vaccination.

Acute thrombocytopenia developed 3 weeks after the vaccination of a child with leukemia with V-Z vaccine. This is the first case of postvaccinal thrombocytopenia to be reported following V-Z vaccine. The risk to leukemic children of postvaccinal thrombocytopenia would appear to be less than 1%.