Clinico-pathological and Immunophenotype Analysis of Hairy Cell Leukemia Cases – A Single Institute Experience

Background: Hairy cell leukemia is a mature lymphoid B cell disorder, characterized by hairy cells, a specific genetic profile, different clinical course and the need for an appropriate treatment. It is seen primarily in elderly, characterized by a triad of splenomegaly, pancytopenia and monocytopenia. Aim: To evaluate clinicopathologically and with immunophenotyping hairy cell leukemia cases received at our institute in conjunction with similar studies. Material and methods: This is a retrospective study which included 7 cases over a period of 3 years (2019-2021) confirmed on morphology and flow cytometry. Results: The study revealed 7 cases which showed patients with age ranging from 34-65 years. M:F ratio was 6:1. Two cases were covid positive (28.5%). Most of the cases presented with fever, weakness (28.5%). Splenomegaly was seen in three of the cases (42.6%). Laboratory investigations revealed anemia in 71% cases, leucopenia in 56.8%, lymphocytic prominence in 100% and pancytopenia in 14.2%. One patient presented with leukocystosis (14.2%). Marrow was hemodiluted and aparticulate in 3(42.6%) cases. Hairy cells were seen on morphology of peripheral smear and marrow aspirate. On flow cytometry, CD5 negative in all cases (100%), CD10 positive in 2(28.5%) and CD23 in 2 cases (28.5%). Few cases confirmed BRAF v600e mutations. Conclusion: Unusual findings like leukocytosis, absence of spleen, presence of lymphadenopathy can be present in hairy cell leukemia. Classical fried egg appearance in trephine biopsy may not be afeature in all the cases. CD123 is expressed in covid patients unlike other studies and further research is needed to establish the loss of CD123 in covid patients.

Study of Salivary Gland Neoplasms.

Tumors of the salivary glands are uncommon and they present a great variety of histological types with many structural variation. They have a low incidence and their anatomical relationship especially that of the parotid gland to the facial nerve make their management challenging. The present study was carried out to find out the prevalence of salivary gland neoplasms, to see associated conditions like sialadenitis, sialolithiasis coexisting with salivary gland neoplasms and to do cytohistopathological correlation. Total 96 cases were collected of which 66.7% were benign and 33.3% were malignant. In 81 (84.3%) cases tumors occurred in major salivary gland while 15 (15.6%) cases in minor salivary gland. Among major salivary gland, parotid was the commonest site and in minor salivary glands, palate was the commonest site followed by lips. Pleomorphic adenoma was the commonest benign tumor and adenoid cystic carcinoma was the commonest malignant tumor followed by mucoepidermoid tumor. There was one case of synovial sarcoma. Associated pathology like sialadenitis and sialolithiasis were present in 16 benign and 5 malignant tumors. Cytohistocorrelation was seen in 63 out of 64 (98.4%) benign and 16 out of 18 (88.8%) malignant tumors.

Leukemic phase of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) is a distinct type of CD30+ T/null-cell non-Hodgkin's lymphoma that frequently involves nodal and extranodal sites. The presence of leukemic phase in ALCL is extremely rare and occurs exclusively with ALK1-positive ALCL. We describe two patients with ALK1-positive ALCL who developed a leukemic phase with rapid progression of the disease. Immunophenotypic pattern assessed on peripheral blood by flow cytometry revealed CD45, CD30, and CD25 positivity in both cases but NPM-ALK1 was expressed in only one case. Both patients developed leukemic phase as a terminal event of the disease and we share the immunophenotypic features of both cases.