ABSTRACT
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.
Subject(s)
Humans , Male , Aged , Leukemia, Lymphocytic, Chronic, B-Cell , Leukemia, Prolymphocytic , Leukemia, Lymphoid , Leukemia, Prolymphocytic, B-Cell , Rare Diseases , LymphadenopathyABSTRACT
<p><b>OBJECTIVE</b>To investigate the clinicopathologic features, diagnosis, differential diagnosis and the prognosis of hairy cell leukemia (HCL).</p><p><b>METHODS</b>Fifteen splenectomy specimens of HCL patients were investigated retrospectively using HE and immunohistochemistry in correlation with the follow-up information.</p><p><b>RESULTS</b>(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.</p><p><b>CONCLUSIONS</b>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.</p>
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Annexin A1 , Metabolism , Antigens, CD20 , Metabolism , CD11c Antigen , Metabolism , CD79 Antigens , Metabolism , Diagnosis, Differential , Follow-Up Studies , Ki-67 Antigen , Metabolism , Leukemia, Hairy Cell , Metabolism , Pathology , General 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 , Retrospective Studies , Spleen , Pathology , Splenectomy , Survival RateABSTRACT
To investigate the clinical, cellular morphology, immunophenotype, and cytogenetic characteristics of acute myeloid leukemia (AML) which are very similar to the morphological characteristics of prolymphocytic leukemia (PLL), the morphological features of bone marrow cells from patient were observed by light microscope, the immunophenotypes were detected by flow cytometry, the karyotypes were analyzed by conventional cytogenetic method, the hybridization signals were determined by fluorescence in situ hybridization. The results indicated that the clinical features were in accordance with acute leukemia and the immunophenotyping results showed malignant cells originated from myeloid lineage, while the cytomorphology analysis showed that the blastic cells were more like the lymphoid lineage. Trisomy 8 was found in the patient by cytogenetic study, the patient did not show good response to chemotherapy. In conclusion, acute leukemia has high heterogenicity, which could be defined as AML, but more like lymphocytic origination by morphological study. Immunophenotyping analysis could contribute to the final diagnosis of malignant cells.
Subject(s)
Adult , Humans , Male , Bone Marrow Examination , Cytogenetics , Immunophenotyping , Leukemia, Myeloid, Acute , Allergy and Immunology , Pathology , Leukemia, Prolymphocytic , Allergy and Immunology , PathologyABSTRACT
T- cell Prolymhocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy that is usually reported in the elderly and follows an aggressive course. A 68 year old male presented with a history of weakness and weight loss of two months duration. Clinical examination revealed pallor, enlarged cervical and axillary lymph nodes and splenomegaly. He also had a maculo- papular skin rash. There was marked leucocytosis, anemia and thrombocytopenia (WBC 445 x 103 sub/ml, Hb 8.5 gm/dl, Platelet 25 x 103 sub/microl) with 60% prolymphocytes in the peripheral blood. Bone marrow was hypercellular with an excess of prolymphocytes. Flow cytometric analysis of the bone marrow showed positivity for CD2, CD3, CD4, CD5 and CD 7. T- PLL is a rare T cell disorder with characteristic clinical and laboratory features. Currently, no optimal treatment exists although there has been some success with 2'- deoxycoformycin or Campath-1H.
Subject(s)
Aged , Bone Marrow Cells/pathology , Diagnosis, Differential , Humans , Leukemia, Prolymphocytic/blood , Male , Weight LossABSTRACT
Prolymphocytic leukaemia is a rare subtype of chronic lymphocytic leukaemia. Three such cases are reported here along with clinical details. All these cases were seen in males above 5th decade of life. These patients showed moderate to massive splenomegaly, inconspicuous lymphadenopathy in two cases and one with minimal lymphadenopathy. Peripheral smear showed high leucocyte count with more than 55% of prolymphocytes. Bone marrow aspiration showed diffuse involvement; and in one with minimal lymphadenopathy, lymph node aspiration showed prolymphocytes. All the three patients died within a year after diagnosis.
Subject(s)
Aged , Antineoplastic Agents/therapeutic use , Diagnosis, Differential , Fatal Outcome , Humans , Leukemia, Prolymphocytic/diagnosis , Male , Middle AgedABSTRACT
Prolymphocytic leukaemia is a rare subtype of chronic lymphocytic leukaemia. Three such cases were reported here along with clinical details. All these cases were seen in males above 5th decade. These patients showed moderate to massive splenomegaly, inconspicuous lymphadenoapthy in two cases and one with minimal lymphadenopathy. Peripheral smear showed high leukocyte count with more than 55% of prolymphocytes. Bone marrow aspiration showed diffuse involvement and in one with minimal lymphadenopathy, lymphnode aspiration showed prolymphocytes. All the three patients died within a year after diagnosis.
Subject(s)
Aged , Bone Marrow/pathology , Humans , Leukemia, Prolymphocytic/blood , Lymphatic Diseases/pathology , Male , Middle Aged , Splenomegaly/pathologyABSTRACT
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.
Subject(s)
Humans , Middle Aged , Bone Marrow , Coombs Test , HLA-DR Antigens , Immunoglobulins , Immunophenotyping , Leukemia , Leukemia, Prolymphocytic , Leukemia, Prolymphocytic, B-Cell , Leukocytosis , Lymphatic Diseases , Lymphocytosis , SplenomegalyABSTRACT
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.
Subject(s)
Humans , B-Lymphocytes , Chlorambucil , Drug Therapy , Hepatomegaly , Leukemia , Leukemia, Lymphocytic, Chronic, B-Cell , Leukemia, Prolymphocytic , Lymph Nodes , Physical Examination , Prednisolone , Prognosis , SplenomegalyABSTRACT
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.
Subject(s)
Female , Humans , Middle Aged , Abdominal Pain , Arthritis , B-Lymphocytes , Basophils , Biopsy , Bone Marrow , Chromatin , Cytoplasm , Drug Therapy , Follow-Up Studies , Gingival Hypertrophy , Hysterectomy , Immunohistochemistry , Leukemia, Prolymphocytic , Leukemia, Prolymphocytic, B-Cell , Leukocytes , Lymphatic Diseases , Lymphocytes , Lymphoproliferative Disorders , Ovary , Phenotype , Physical Examination , Splenomegaly , UterusABSTRACT
Prolymphocytic leukemia (PLL) is a member of chronic lymphoproliferative disorders with relatively distinct clinical, morphologic, immunologic and prognostic features. The diagnosis of PLL is determined by more than 55% of prolymphocytes in the peripheral blood. It is characterized by leukocytosis, massive splenomegaly with little or no lymphadenopathy, and male prevalence. In immunophenotyping, the majority (80%) of the cases express B cell markers and the rest (20%), T cell type. We experienced a case of B-PLL. The patient was a 65 year-old man who presented with marked leukocytosis (110.5x109/L) and 78% of characteristic prolymphocytes in the peripheral blood. The bone marrow aspirate showed 13.6% of prolymphocytes and coarse granular positivity of prolymphocytes in PAS stain. The immunophenotyping of the leukemic prolymphocytes revealed the positivity of surface immunoglobulin (IgM, lambda type), HLA- DR and CD19. The monoclonal gammopathy (IgM, lambda type) was also detectable in the patient's serum.
Subject(s)
Aged , Humans , Male , Bone Marrow , Diagnosis , Immunoglobulins , Immunophenotyping , Leukemia , Leukemia, Prolymphocytic , Leukocytosis , Lymphatic Diseases , Lymphoproliferative Disorders , Paraproteinemias , Prevalence , SplenomegalyABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Biopsy , Bone Marrow , Cytoplasm , Germinal Center , Leukemia, B-Cell , Leukemia, Lymphocytic, Chronic, B-Cell , Leukemia, Prolymphocytic , Lymph Nodes , Lymphatic Diseases , Lymphocytes , Lymphocytosis , Lymphoma, Follicular , Lymphoma, Mantle-Cell , Membranes , PhenotypeABSTRACT
We present the clinical and laboratory features of 2 patients with B prolymphocitic leukemia. Both are females of the fith and seventh decade of life. One had the classical clinical picture of massive splenomegaly and a high white cell count, with characteristic prolymphocytes and the other was asymptomatic, with a low white cell count. The cells were positive to B cell lineage reagents with strong surface immunoglobuline (Ig) and unreactive to T cell antibodies. Analysis of Ig genes at the DNA level demonstrated that both cases had heavy-chain gene rearrangements, confirming the B-cell origin. These are the first patients of prolymphocytic leukemia described in Chile
Subject(s)
Middle Aged , Leukemia, Prolymphocytic/diagnosis , DNA Probes/analysis , Immunophenotyping/methods , Genetic Markers/immunologyABSTRACT
The clinical histopathological and histochemical features of ten patients with prolymphocytic leukemia were reviewed. Seven of the patients had clinical evidence of massive splenomegaly at the time of the diagnosis. None of the patients had significant lymph nodes enlargement. The initial leucocyte count was elevated in all patients and was greater than 100 * 10[9]/1 in eight of them. The absolute prolymphocyte count range from 18.4 to 395.3 * 10[9]/1 and was greater than 100 * 10[9]/1 in eight patients