Hairy cell leukemia (leukemic reticuloendotheliosis). I. A clinical pathologic study of 21 patients. [21 patients]

Journal Article · Wed May 01 04:00:00 EDT 1974 · Cancer; (United States)

DOI:https://doi.org/10.1002/1097-0142(197405)33:5<1399::AID-CNCR2820330526>3.0.CO;2-E· OSTI ID:5334483

Burke, J S; Byrne, Jr, G E; Rappaport, H

The clinical and pathologic findings in 21 patients with hairy cell leukemia (leukemic reticuloendotheliosis) are reviewed. The disease predominantly involved males, and was characterized by a chronic course with an insidious onset and marked splenomegaly. A hypersplenic syndrome with pancytopenia, ''hairy cells'' in the peripheral blood, and frequent ''dry taps'' on bone marrow aspiration were common features. Only one patient in this series was leukemic at the onset of disease. Pathologically, the changes in the spleen were distinct from those in other leukemias and malignant lymphomas, and consisted of a diffuse heavy infiltration of the red pulp cords and engorgement of the sinuses by a uniform population of mononuclear cells without mitoses. These cells were not identifiable as either monocytes or lymphocytes, although the pattern of infiltration was more consistent with histiocytic or monocytic than with lymphocytic proliferation. Infiltrates of similar cells were found in the bone marrow, lymph nodes, and livers of many patients early in the course of the disease. Despite this diffuse organ involvement, only 4 patients in this series died, and long survival was common. Splenectomy appeared to be the treatment of choice, whereas vigorous chemotherapy was contraindicated. Accurate diagnosis by recognition of the histologic criteria is therefore essential.

• Research Organization: Univ. of Chicago
• OSTI ID: 5334483
• Journal Information: Cancer; (United States), Journal Name: Cancer; (United States) Vol. 33:5; ISSN CANCA
• Country of Publication: United States
• Language: English

Related Subjects

550900* -- Pathology
59 BASIC BIOLOGICAL SCIENCES
DISEASES
DOCUMENT TYPES
HEMIC DISEASES
IMMUNITY
IMMUNOLOGY
LEUKEMIA
MALES
NEOPLASMS
PATHOLOGY
REVIEWS