Case Report Amee Soni *, Chandni Gadara*, Preeti Jhaveri**, Anjali Goyal***, C. K. Shah **** Hairy Cell Leukemia- A Case Report * Resident ** Assistant Professor *** Associate Professor **** Professor and Head, Pathology Department, Smt. N.H.L Municipal Medical College, Ahmedabad, Gujarat, India Correspondence : Dr. Amee Soni E-mail : [email protected]Introduction : Hairy cell leukemia is recognized as an entity by the world health organization (WHO 2008) and the 2016 revision of the WHO classification of lymphoid neoplasm.HCL is rare chronic B-cell disorder, accounting for 2% of Non- Hodgkin lymphomas and occurs more frequently in men with an overall incidence of 2.9 per million per year for men and 0.6 per million (1) per year for women in the united states. The etiology of HCL is unknown, although case control studies have identified possible relationships to farming (including exposure to farm animals, commercial herbicides and/or pesticides) and an inverse correlation with (1) smoking. More recently it has been demonstrated that most hairy cells contain a mutated active form of the BRAF gene(V600E) which may be used as a diagnostic (2) tool and a potential target for therapy. Complete blood count and careful review of the peripheral blood smear are the first step in very characteristic morphological identification of hairy cells. Diagnosis is achieved by studies on peripheral blood including flow cytometry and review of peripheral blood smear along with bone marrow biopsy. The flow cytometry markers positive in hairy cell leukemia include CD 11c, CD 25, Abstract : Hairy cell leukemia is rare chronic B cell malignancy that involves the bone marrow, spleen and peripheral blood. Median age of diagnosis is approximately 55 years with significant higher incidence in Men and male to female ration of 4:1. The disease is characterized by the presence of typical hairy cells in peripheral blood and bone marrow, pancytopenia and a variable degree of splenomegaly. Classically patient present with weakness, fatigue, symptoms related to pancytopenia and splenomegaly. The etiology of hairy cell leukemia not well elucidated. However previous exposure to various chemicals may play a role in it's development. Most cases are postulated to be derived from V600E BRAF gene mutation of late activated memory B cell. Key Words : B-cell lymphoid leukemia, Hair like projection, Hairy cell leukemia, V600E BRAF mutation :: 32 :: CD 103, along with typical B- cell marker such as CD19, (2) CD20 or CD22. Hairy cells can be identified in Romanowsky-stained peripheral blood films from approximately 90% of patients as mononuclear cells that are usually 1 to 2 times the size of mature lymphocyte. Case report: An old female patient, aged 40 years, presented to out- patient department with chief complain of abdominal pain since 2 years. Patient was relatively asymptomatic before 2 years. Then she develops abdominal pain which was diffuse, insidious in nature and associated with nausea, constipation, vomiting and intermittent fever. On clinical examination spleen was palpable. USG findings showed hepatosplenomegaly. On peripheral blood smear WBC series showed lymphocytosis with large atypical lymphocytes showing irregular cell membrane and moderate basophilic cytoplasm. Many cells show hair-like cytoplasmic processes and few show polar villous cyoplasmic processes. Nuclei are round to oval with clumped chromatin and indistinct nucleoli. Patient also has anemia and thrombocytopenia. Findings are suggested possibility of hairy cell leukemia. Bone marrow biopsy was performed which shows hypercellular marrow. Occasional megakaryocytes were seen. It shows significantly increased lymphoid cells, approximately 58% with many showing hairy cell projection. Erythroid series show normoblastic maturation. Granulocytic series is suppressed. And the diagnosis of hypercellular marrow involved by hairy cell leukemia was made. GCSMC J Med Sci Vol (IX) No (I) January-June 2020
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Case Report
Amee Soni *, Chandni Gadara*, Preeti Jhaveri**, Anjali Goyal***, C. K. Shah ****
Hairy Cell Leukemia- A Case Report
* Resident
** Assistant Professor
*** Associate Professor
**** Professor and Head, Pathology Department, Smt. N.H.L
Municipal Medical College, Ahmedabad, Gujarat, India