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.
Bone Marrow ; Coombs Test* ; HLA-DR Antigens ; Humans ; Immunoglobulins ; Immunophenotyping ; Leukemia ; Leukemia, Prolymphocytic* ; Leukemia, Prolymphocytic, B-Cell ; Leukocytosis ; Lymphatic Diseases ; Lymphocytosis ; Middle Aged ; Splenomegaly

Prolymphocytic leukemia (PL) is usually derived from B cells and shares some features with chronic lymphocytic leukemia (CLL), but it is clearly a distinct entity and defined to have more than 55% prolymphocytes. Chronic lymphocytic leukemia/prolymphocytic leukemia (CLL/PL) is a mixed type of CLL and is defined to have 11~55% prolymphocytes with intermediate features between CLL and PL. We experienced two different cases of leukemia PL and CLL/PL. On physical examination, PL (stage II, B) patient showed multiple cervical lymph node enlargement and 5cm sized splenomegaly and hepatomegaly. But CLL/PL (stage III, C) patient showed 10cm sized splenomegaly and no lymph node enlargement. On immunological phenotyping, surface markers showed 72% CD5 (+), 85% CD19 (+), and 40% SmIg (+) in PL patient and 3% CD5 (+), 90% CD19 (+) and SmIg (-) in CLL/PL patient. PL patient was refractory to chlorambucil and prednisolone chemotherapy and showed poor prognosis. CLL/PL patient did not show remarkable response to chlorambucil and prednisolone therapy.
B-Lymphocytes ; Chlorambucil ; Drug Therapy ; Hepatomegaly ; Humans ; Leukemia* ; Leukemia, Lymphocytic, Chronic, B-Cell ; Leukemia, Prolymphocytic ; Lymph Nodes ; Physical Examination ; Prednisolone ; Prognosis ; Splenomegaly

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Leukemia, Prolymphocytic, B-Cell ; diagnosis ; Male ; Middle Aged ; Retrospective Studies

Recently, we experienced a patient with a B cell leukemia which could not be classified as chronic lymphocytic leukemia (CLL), prolymphocytic leukemia (PL) or follicular lymphoma in leukemic phase. He is a 58-year-old male and suffered from progressive lymphadenopathy at both inguinal and cervical areas. His blood film showed lymphocytosis (79%), and the cells were heterogeneous in size and shape. Twenty-five percent of the lymphocytes had nuclear irregularities or cleavages unlike the lymphocytes of CLL In which the cells often shows monomorphic features, small size with scanty cytoplasm and round nucleus. The bone marrow trephine biopsy specimen showed a diffuse pattern of infiltration of atypical Iymphoid cells. The lymph node histology showed atypical lymphoid colls proliferated as wide mantles around non-neoplastic appearing germinal centers. The immunophenotype of circulating lymphocytes in peripheral blood showed strong reactivity with CD5, CDl9 and CD20 without expression of CD2, CD3, CD7, CD10, CD22 and CD23. Although this case resembled CLL, the laboratory features showed major differences, notably in the Peripheral blood morphology, histology Patterns and the membrane Phenotype. By combining these data, we diagnosed this case as a leukemic phase of mantle cell lymphoma.
Biopsy ; Bone Marrow ; Cytoplasm ; Germinal Center ; Humans ; Leukemia, B-Cell ; Leukemia, Lymphocytic, Chronic, B-Cell ; Leukemia, Prolymphocytic ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphocytosis ; Lymphoma, Follicular ; Lymphoma, Mantle-Cell* ; Male ; Membranes ; Middle Aged ; Phenotype

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.
Abdominal Pain ; Arthritis ; B-Lymphocytes* ; Basophils ; Biopsy ; Bone Marrow ; Chromatin ; Cytoplasm ; Drug Therapy ; Female* ; Follow-Up Studies ; Gingival Hypertrophy ; Humans ; Hysterectomy ; Immunohistochemistry ; Leukemia, Prolymphocytic ; Leukemia, Prolymphocytic, B-Cell* ; Leukocytes ; Lymphatic Diseases ; Lymphocytes ; Lymphoproliferative Disorders ; Middle Aged ; Ovary ; Phenotype ; Physical Examination ; Splenomegaly ; Uterus

<b>OBJECTIVEb>To investigate the clinicopathologic features, diagnosis, differential diagnosis and the prognosis of hairy cell leukemia (HCL).

<b>METHODSb>Fifteen splenectomy specimens of HCL patients were investigated retrospectively using HE and immunohistochemistry in correlation with the follow-up information.

<b>RESULTSb>(1) The male to female ratio was 2.75:1, age ranged from 36 to 68 years with a median of 47 years. The most consistent clinical feature at presentation was marked splenomegaly (100%). Other symptoms included anemia (80.0%), thrombocytopenia (60.0%), leucocytosis (53.3%), pancytopenia (20.0%) and the absence of B-symptom. (2) The proportion of hairy cells was (14.6 +/- 7.2)% in periphery blood and (47.3 +/- 23.8)% in bone marrow. The positive rate of TRAP assay was 62.5% in bone marrow; 85.7% for TPA test and the detection rate for RLC was 25% by transmission electric microscopy. The frequency of bone marrow involvement was 100%. (3) The average weight of 15 spleens was (3012 +/- 1974) g. The size of 6 spleens ranged from 16 cm x 10 cm x 5 cm to 32 cm x 20 cm x 14 cm. The white pulp of spleen showed a characteristic atrophy feature or even absent due to leukemic infiltration, predominantly involving the red pulp with some sinusoidal pattern. "Blood pool" change was an infrequent feature (3/15 cases). The nuclei of leukemic cells were round (13 cases) or bean-shaped (2 cases), nucleoli inconspicuous or disappeared. The abundant cytoplasm and prominent cell border resulted in a "fried egg" appearance. By immunohistochemistry, leukemic cells were positive for CD45RA, CD20, PAX-5, CD25, CD11c, Annexin A1 and cyclinD1, but negative for CD3 and CD43. (4) 13 cases (86.7%) have been followed-up and all are alive. Among them, 9 cases are living well more than 5 years and 7 more than 10 years.

<b>CONCLUSIONSb>Splenomegaly is frequently the first manifestation of patients with HCL and occurred predominantly in the middle to elderly adults. Definite diagnosis of HCL requires a combined histological and immunohistochemical assessment of the splenectomy specimen, bone marrow biopsy and aspirate.

Adult ; Aged ; Annexin A1 ; metabolism ; Antigens, CD20 ; metabolism ; CD11c Antigen ; metabolism ; CD79 Antigens ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Ki-67 Antigen ; metabolism ; Leukemia, Hairy Cell ; metabolism ; pathology ; surgery ; Leukemia, Lymphocytic, Chronic, B-Cell ; metabolism ; pathology ; Leukemia, Prolymphocytic ; metabolism ; pathology ; Leukocyte Common Antigens ; metabolism ; Lymphoma, B-Cell, Marginal Zone ; metabolism ; pathology ; Lymphoma, Follicular ; metabolism ; pathology ; Lymphoma, Mantle-Cell ; metabolism ; pathology ; Male ; Middle Aged ; Retrospective Studies ; Spleen ; pathology ; Splenectomy ; Survival Rate