De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

A case of de novo B - cell prolymphocytic leukemia associated with false - positive direct antiglobulin test / 대한내과학회지

De novo B-cell prolymphocytic leukemia (B-PLL) is a distinct clinicopathologic entity that was first described in 1974 by Galton et al. B-PLL is characterized by marked lymphocytosis with predominance of prolymphocytes, often massive splenomegaly, minimal lymphadenopathy, often aggressive clinical course and frequently poor prognosis.We experienced a case of B-PLL associated with false-positive direct antiglobulin test. The patient was 52 year-old man who presented with marked leukocytosis (160.2x103/(mu)L) and 60% of characteristic prolymphocytes in the peripheral blood. The bone marrow aspirate showed dry tap and the PAS stain of peripheral blood smear showed positivity of prolymphocytes. The immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA-DR, CD19 and CD5.

B-cell Prolymphocytic Leukemia Involving Entire Female Genital Tract: A case report

Prolymphocytic leukemia is a chronic lymphoproliferative disorder, characterized by prominent splenomegaly, prolymphocytes accounting for more than 55% of circulating lymphocytes, no significant peripheral lymphadenopathy and short term survival with terminal fatal multi-organ failure. We report a case of B-cell prolymphocytic leukemia in a 57-year-old woman who presented with easy bruising and arthritis for 1 year and low abdominal pain for 2 months. Physical examination revealed gingival hypertrophy and mild splenomegaly. On peripheral blood smears the leukocytes were markedly increased in number due to leukemic cells that count about 62% of leukocytes. The bone marrow aspiration smear and biopsy revealed diffuse infiltration of medium to large prolymphocytes having moderate amount of basophilic cytoplasm, round to oval nuclei with coarse chromatin, and prominent nucleoli. Abdominal pain aggravated despite chemotherapy, and pelvic computed tomography (CT) revealed a huge lobular pelvic mass which had increased in size on the follow-up CT. Total hysterectomy with bilateral adnexectomy was performed. Microscopic findings included massive infiltration of prolymphocytic cells in the uterus, upper vaginal wall, bilateral ovaries, and bilateral mesosalpinges. On immunohistochemistry, the leukemic cells showed B cell gamma light chain phenotype.