유모세포 백혈병 -1예 보고-
Hairy Cell Leukemia -A case report-
소속 상세정보
정재걸/Jae Gul Chung
공경엽/최기영/유은실/이인철/박건춘/김상희/Gyung Yub Gong/Ghee Young Choe/Eun Sil Yu/In Chul Lee/Kun Choon Park/Sang Hee Kim
KMID : 0357919940280060675
Abstract
유모세포 백혈병(hairy cell leukemia)은 중년 남성에서 주로 발생하는 드문 림프세포증식
성 질환으로서 말초혈액, 골수, 비장, 간등을 잘 침범한다. 국내에서는 1979년 복부종괴를 주
소로한 2예만이 보고된 적이 있는 드문 질환이며, 서양에서도 그 빈도가 높지 않다. 저자들
은 복부종괴를 주소로 한 유모세포 백혈병 1예를 경험하여 임상적 혈액학적, 조직학적 및
전자현미경적 소견과 함께 보고하는 바이다.
#초록#
Hairy cell leukemia is an uncommon lymphoreticular disorder which primarily involves
bone marrow, spleen, and peripheral blood. Patients, mostly men, present with
splenomegaly and pan-cytopenia usually.
A 62-year-old man was admitted with an abdominnal mass which had grown slowly
for 20 years. On physical examination, an enlarged spleen was palpated without
tenderness. An abloom mal CT scan showed a diffusely enlarged spleen, which
measured 20 ㎝ in greatest dimension. In the peripheral blood, many atypical
lymphocytes with abundant, delicate, surface projections were noted. They had
tartrate-resistant acid phosphatase(TRAP) activity. Thrombocytopenia(60,000/㎣) was
observed in the complete blood counts, Other laboratory data were within normal limits.
He underwent splenectomy.
The submitted spleen measured 26×15×5 ㎝ and weighed 2150 gm. It was well
encapsulated and the outer surface was smooth. Cut surfaces were diffusely dank-red.
W kite and red pulps were indistinct. There was no mass-like lesion. Microscopically,
the spleen consisted of monotonous mononulcear cells which involved red pulp. The
white pulp was diminished, and could be barely recognized. Cells had small round nuclei
and abundant cytoplasm. Ultrastructurally, cells with numerous slender surface
projections were noted.
In Korea, hairy cell leukemia Is exceedingly rare. We report a case of hairy cell
leukemia with characteristic pathologic features of spleen as well as those of peripheral
blood. (Korean J Pathol 1994; 28: 675∼677)
키워드
Hairy cell leukemia; Spleen;
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