The medical care of young adults: the practice of ephebiatrics.
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Biomedical subjects
Publications and source records attributed to D M Komp.
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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. Of the various chemotherapeutic regimens that have been tried so far, a combination of vinca alkaloid, alkylating agent, and corticosteroid appears to be the most effective.
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. Adoption of a uniform clinical/laboratory approach to these children, coupled with agreed-upon steps to the pathologic identification of LCH, should result in a better understanding and ultimately better and more rational therapies for the disease.
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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). Serum SIL-2R is a marker of disease activity that has the potential to identify infants at risk for the inherited form of the disease before the disease is clinically expressed.
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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.
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. There was no apparent clinical significance to the anticoagulant. It did, however, confuse the interpretation of coagulation factor assays based on animal substrate plasmas.
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%. Note added in proof: The patient remained seronegative until the eighth month following vaccination. Her FAMA titer then rose to 1:32, probably as a result of exogenous reinfection with V-Z virus.
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