Letterer-Siwe病

LETTERER-SIWE'S DISEASE

　1924年，Letterer^1）により，および1933年Si-we^2）によつて報告されて以来，数多くのLette-rer-Siwe病の報告があり，わが国においても1970年までに80例を越す報告をみることができる．

　本症は病理組織学的に，広汎に細網内皮細胞が増殖する疾患の一つとしてとらえられているが，細網内皮症の概念が未だに混沌としており，その病因に関しては定説が無い．

A case of this disease in a 8-month-old girl was reported, which seemed to start at the age of 3 months.

At her hospitalization, fever (38.4 C), severe anemia, marked hepatosplenomegaly, and external otitis were noted.

Biopsy specimen of the skin eruption showed a dense infiltration of large round cells in the dermis which was negative by the fat stain and contained the Birbeck granules with electron microscopy. Roent-genogram of the skull showed several punched-out figures.

Levels of the srum immunoglobulins were 2 folds of the normal level, which were as follows, IgG, 2,000 mg/dl; IgA, 220 mg/dl and IgM, 105 mg/dl. Results of DNCB skin sensitization for cellular immunity and the blast-like cell formation rate of lymphocyte by PHA were within normal limits. Her clinical symptomes were improved markedly by intravenous injection of Daunomycin (0.5-1.0 mg/kg daily, and total doses of 94.5 mg). Then the maintenance doses of beta-methazone (1.0 mg/day, p.o.) have been followed. Her general condition was excellent 13 months after the onset of the disease when she was checked again.

During the remission, serum immunoglobulin levels returned to the normal (IgG, 1,000 mg/di; IgA, 93 mg/d1 and IgM, 75 mg/dl). Relationships between the number of lymphocyte in the peripheral blood and serum immunoglobulin levels were not proved. No difference in the blast-like cell formation rate of lymphocyte was revealed before and after the remission.