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

Abstract

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.

Rare lymphoid malignancies of the breast: a report of two cases illustrating potential diagnostic pitfalls

“…Hairy cell leukemia typically involves bone marrow and spleen but may also involve other sites including the central nervous system, gastrointestinal and urogenital tracts, heart, lungs, skeletal muscle, skin, thymus, and thyroid [28–32]. Breast involvement, either at the time of diagnosis or later in the disease course, has not been reported to our knowledge.…”

Malignant histiocytosis (histiocytic medullary reticulosis) with spindle cell differentiation and tumour formation.

“…Problems in the differential diagnosis of malignant histiocytosis have been discussed in detail elsewhere (Byrne and Rappaport, 1973;Burke et al 1974;Wamke et al, 1975). Among the conditions which have to be distinguished from malignant histiocytosis are Hodgkin's disease, histiocytic lymphoma (reticulum cell sarcoma), sinus histiocytosis with massive lymphadenopathy, hairy cell leukaemia (leukaemic reticuloendotheliosis), and histiocytosis X.…”

Small B-cell lymphomas of the spleen: how to tell them apart

“…On microscopic examination, red pulp sinuses and cords are diffusely infiltrated by monomorphous round to oval cells with smooth nuclear contours, open chromatin, inconspicuous nucleoli, and abundant clear cytoplasm, leading to expansion of the red pulp sinuses and secondary atrophy of the white pulp (Fig. 4b) [36]. Although not specific to HCL involving the spleen, microscopic erythrocyte-filled spaces, termed "pseudosinuses" or "red blood cell lakes," are characteristically present, which are thought to result from leukemic cell adhesion and subsequent damage to splenic sinus endothelial cells [37].…”