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Hairy cell leukemia—An unusual presentation
Leukemia Research Vol. 19, No. 7, pp. 485-487, 1995. cOpyri&t 43 1995 Elscvier Science Ltd Printed in &cat Britain. All rights reserved 0145-2126/95 $9.50 + 0.00
Pergamon 0145.2126(95)00014-3
CONCISE REPORT HAIRY CELL LEUKEMIA-AN
UNUSUAL PRESENTATION
R. Goyal, S. Bajpai, H. K. Chopra, S. C. Shinde, Y. Badrinath, R. S. Sapre,C. N. Nair and S. H. Advani Departmentof MedicalOncology,TataMemorialHospital,Bombay400 012, India (Received
1 March 1995. Accepted 1 April 1995)
Abstract-Hairy cell leukemia is a chronic lymphoproliferative disorder affecting middle-aged adults, with the median age of 5&55 years. The majority of the patients present with cytopenia. A high count is usually a feature of the hairy cell leukemia variant. We report a case of a 23year-old male who presented with fever and cough of 15 days duration. His peripheral blood count was 63 x 10% His peripheral blood and bone marrow smear showed hairy cells which were positive for tartarate-resistant acid phosphatase stain. Surface markers and electron microscopic study on peripheral blood ruled out hairy cell leukemia variant as a differential diagnosis. Key words: Leukemia,
hairy cell, unusual
presentation.
lymphadenopathy. His respiratory and cardiovascular were normal. Abdominal examination revealed a 5 cm palpable spleen and mild hepatomegaly. His hematological profile revealed a hemoglobin of 8 g/dl, total count of 63 x log/l and platelet count of 125 x log/l. The peripheral blood smear showed 95% of large cells with abundant cytoplasm which was weakly basophilic. The nucleocytoplasmic ratio was low. They showed villous or hairy cytoplasmic projections and a round to oval nucleus. A phase contrast microscopy performed on peripheral blood showed prominent hairy projections. Bone marrow aspiration showed replacement by similar looking cells. Cytochemically these cells were negative for myeloperoxidase, non-specific esterase, Sudan Black B and periodic acid Schiff stain. Acid phosphatasewas positive in 65% cells, 30% of which were tartarate resistant. The bone marrow biopsy showed a diffuse infiltration by hairy cells recognized by their blunt nuclei infiltrating the marrow in a loose fashion with a well-defined rim of cytoplasm that left a clear zone around the cells. Keeping these tidings in mind we thought of a diagnosis of HCL with an early age of presentation. However, due to a high count at presentation an HCL variant could not be ruled out as reported by Catovsky
Introduction Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder of the adult age group which accounts for approximately 2% of all forms of leukemia [ 11.This disease together with B-chronic lymphocytic leukemia (B-CLL) and B-prolymphocytic leukemia (B-PLL) represents one of the main clinical types of B-cell leukemia in humans. HCL affects middle-aged adults with an approximate four fold male predominance.The median ageof patients in most large seriesis between50 and 55 years with a range of 2440 years [2]. Most of the clinical manifestations are a direct result of cytopenia and splenomegaly. The blood counts always reveal variable degrees of cytopenia with 75% of patients having pancytopenia. We report here a caseof hairy cell leukemia in a 23year-old male with a total WBC count of 63 x log/l. Case report The patient, a 23-year-old male presented with complaints of fever with cough of 10-15 days duration. He had no other significant positive history. He was in a good general condition at presentation. On examination he had mild pallor and sternal tenderness with no
Correspondence to: Dr C. N. Nair, Departmentof Medical Oncology,Tata MemorialHospital,Bombay400 12, India.
[3,41.
Surface marker studieswere carried out on peripheral 485
486
R. Goyalet
al.
Fig. 1. Electron microphotograph of hairy cell showing broad-based multiple cytoplasmic villi of variable sizes and shape, irregular nuclear outline and an evenly dispersednuclear chromatin throughout the nuclear matrix (magnification 12,000 x 6).
Fig. 2. Electron microphotographof hairy cell showing a ribosomal-lamellar complex (magnification 20,000 x 6).
Hairy cell leukemia
blood using immunofluorescentand peroxidaseand antiperoxidasetechniques.These cells were positive for the HCL marker (DBA44), CD22 and CD25 An ultrastructural study carried out on these cells showed that the cells were round to oval with a low nucleocytoplasmic ratio. The nucleus was central with an irregular nuclear outline. In some cells the nucleus was indented. The nuclear chromatin was evenly dispersed throughout the nuclear matrix (Fig. 1). Ribosomal-lamellar complexes and multiple cytoplasmic villi with broad base and variable size and shape were seen(Fig. 2). However, no nucleoli were noted. Thus, the differential diagnosis of HCL variant was ruled out and a diagnosis of HCL with an early age of presentation was established.
487
References 1. Catovsky D. & Foa R. (1990) Hairy cell leukemia. In The Lymphoid Leukemia, p. 156. Butterworth, London. Charles E.H. (1993) Hairy cell leukemia, malignant histiocytosis, and related disorders. In W&robe Clinical Hematology (Lee G.A., Bithell T.C., Foerster J., Athens J.W. & Lukens J.N., Eds), p. 2170. Lea and Febiger, Philadelphia. Bajpai S., Shinde S.C.. Badrinath Y., Dhond S. R., Chougule A. B., Sapre R. S., Barbhaya S. A., Ashok Kumar M. S., Chopra H. K., Nair C. N., Soman C. S. & Advani S. H. (1994) Hairy cell leukemia: laboratory review of 15 cases. Ind. J. Haematol. and Blood. Transf: 12(3), 117. 4. Catovsky D., O’Brien M. & Melo J.V. (1984) Hairy cell leukemia variant: an intermediate disease between hairy cell leukemia and B prolymphocytic leukemia. Semin. Oncol. 11(4), 362.
Pergamon 0145.2126(95)00014-3
CONCISE REPORT HAIRY CELL LEUKEMIA-AN
UNUSUAL PRESENTATION
R. Goyal, S. Bajpai, H. K. Chopra, S. C. Shinde, Y. Badrinath, R. S. Sapre,C. N. Nair and S. H. Advani Departmentof MedicalOncology,TataMemorialHospital,Bombay400 012, India (Received
1 March 1995. Accepted 1 April 1995)
Abstract-Hairy cell leukemia is a chronic lymphoproliferative disorder affecting middle-aged adults, with the median age of 5&55 years. The majority of the patients present with cytopenia. A high count is usually a feature of the hairy cell leukemia variant. We report a case of a 23year-old male who presented with fever and cough of 15 days duration. His peripheral blood count was 63 x 10% His peripheral blood and bone marrow smear showed hairy cells which were positive for tartarate-resistant acid phosphatase stain. Surface markers and electron microscopic study on peripheral blood ruled out hairy cell leukemia variant as a differential diagnosis. Key words: Leukemia,
hairy cell, unusual
presentation.
lymphadenopathy. His respiratory and cardiovascular were normal. Abdominal examination revealed a 5 cm palpable spleen and mild hepatomegaly. His hematological profile revealed a hemoglobin of 8 g/dl, total count of 63 x log/l and platelet count of 125 x log/l. The peripheral blood smear showed 95% of large cells with abundant cytoplasm which was weakly basophilic. The nucleocytoplasmic ratio was low. They showed villous or hairy cytoplasmic projections and a round to oval nucleus. A phase contrast microscopy performed on peripheral blood showed prominent hairy projections. Bone marrow aspiration showed replacement by similar looking cells. Cytochemically these cells were negative for myeloperoxidase, non-specific esterase, Sudan Black B and periodic acid Schiff stain. Acid phosphatasewas positive in 65% cells, 30% of which were tartarate resistant. The bone marrow biopsy showed a diffuse infiltration by hairy cells recognized by their blunt nuclei infiltrating the marrow in a loose fashion with a well-defined rim of cytoplasm that left a clear zone around the cells. Keeping these tidings in mind we thought of a diagnosis of HCL with an early age of presentation. However, due to a high count at presentation an HCL variant could not be ruled out as reported by Catovsky
Introduction Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder of the adult age group which accounts for approximately 2% of all forms of leukemia [ 11.This disease together with B-chronic lymphocytic leukemia (B-CLL) and B-prolymphocytic leukemia (B-PLL) represents one of the main clinical types of B-cell leukemia in humans. HCL affects middle-aged adults with an approximate four fold male predominance.The median ageof patients in most large seriesis between50 and 55 years with a range of 2440 years [2]. Most of the clinical manifestations are a direct result of cytopenia and splenomegaly. The blood counts always reveal variable degrees of cytopenia with 75% of patients having pancytopenia. We report here a caseof hairy cell leukemia in a 23year-old male with a total WBC count of 63 x log/l. Case report The patient, a 23-year-old male presented with complaints of fever with cough of 10-15 days duration. He had no other significant positive history. He was in a good general condition at presentation. On examination he had mild pallor and sternal tenderness with no
Correspondence to: Dr C. N. Nair, Departmentof Medical Oncology,Tata MemorialHospital,Bombay400 12, India.
[3,41.
Surface marker studieswere carried out on peripheral 485
486
R. Goyalet
al.
Fig. 1. Electron microphotograph of hairy cell showing broad-based multiple cytoplasmic villi of variable sizes and shape, irregular nuclear outline and an evenly dispersednuclear chromatin throughout the nuclear matrix (magnification 12,000 x 6).
Fig. 2. Electron microphotographof hairy cell showing a ribosomal-lamellar complex (magnification 20,000 x 6).
Hairy cell leukemia
blood using immunofluorescentand peroxidaseand antiperoxidasetechniques.These cells were positive for the HCL marker (DBA44), CD22 and CD25 An ultrastructural study carried out on these cells showed that the cells were round to oval with a low nucleocytoplasmic ratio. The nucleus was central with an irregular nuclear outline. In some cells the nucleus was indented. The nuclear chromatin was evenly dispersed throughout the nuclear matrix (Fig. 1). Ribosomal-lamellar complexes and multiple cytoplasmic villi with broad base and variable size and shape were seen(Fig. 2). However, no nucleoli were noted. Thus, the differential diagnosis of HCL variant was ruled out and a diagnosis of HCL with an early age of presentation was established.
487
References 1. Catovsky D. & Foa R. (1990) Hairy cell leukemia. In The Lymphoid Leukemia, p. 156. Butterworth, London. Charles E.H. (1993) Hairy cell leukemia, malignant histiocytosis, and related disorders. In W&robe Clinical Hematology (Lee G.A., Bithell T.C., Foerster J., Athens J.W. & Lukens J.N., Eds), p. 2170. Lea and Febiger, Philadelphia. Bajpai S., Shinde S.C.. Badrinath Y., Dhond S. R., Chougule A. B., Sapre R. S., Barbhaya S. A., Ashok Kumar M. S., Chopra H. K., Nair C. N., Soman C. S. & Advani S. H. (1994) Hairy cell leukemia: laboratory review of 15 cases. Ind. J. Haematol. and Blood. Transf: 12(3), 117. 4. Catovsky D., O’Brien M. & Melo J.V. (1984) Hairy cell leukemia variant: an intermediate disease between hairy cell leukemia and B prolymphocytic leukemia. Semin. Oncol. 11(4), 362.