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Authors:
T.Rokni, A.Allali, JFassi Fihri, I.Benjelloun, M. Ait ameur, M.chakour, Morocco
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Abstract:
Hairy cell leukaemia is a rare chronic lymph proliferative disorder characterized by the absence of peripheral lymphadenopathy and the presence of isolated splenomegaly in 75% of cases. The patients are most often neutropenic and monocytopenic and the evolution is dominated by the infectious risk in the untreated subjects. Diagnosis is made by evidence of diffuse infiltration of the spleen and bone marrow by lymphoid B cells: hairy cells. These are particular cells by their hairy appearance with extension towards the peripheral blood. Treatment is by cladribine first, but several other molecules are recommended. Splenectomise remains a last resort with special and limited indications. We report the case of a 35-year-old patient, whose diagnosis was difficult because of the absence of neutropenia and monocytopenia at the beginning of the pathology, as well as inconclusive reports of blood smear and sternal puncture due to non-characteristic morphological appearance of tumour cells. It is the osteomedullary biopsy that guided the diagnosis, which will be confirmed after histological study of the spleen. The management was complicated by clinical-biological worsening even with interferon Alfa treatment, hence the indication for splenectomise, which is rarely performed in hairy cell leukaemia today.
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