Hairy Cell Leukemia (Morphologic and Immunophenotypic Profile)

Hairy Cell Leukemia (HCL) is a lymphoproliferative B cell abnormality dominated by mature lymphocytes with cytoplasmic projections and often misunderstood as Chronic Lymphocytic Leukemia (CLL). Misdiagnosis can be caused by errors in the preparation of peripheral Blood Smear Evaluation (BSE). Immunophenotyping is an option to differentiate HCL from CLL. A 56-year-old female presented with complaints of weakness. Physical examination showed conjunctival anemia 3 3 and hepatosplenomegaly. Hematological test results were as follows: Hb 7.4 g/dL; WBC 131.24x10 /uL; and Plt 61x10 /uL. BSE And Bone Marrow Aspiration (BMA) showed predominantly mature lymphocytes with cytoplasmic projections and suspected CLL with HCL as the differential diagnosis. Immunophenotyping with peripheral blood samples showed CD19+, CD20+, CD79a+, HLA-DR+, CD5-, and CD7- suggesting an increasing mature lymphocytes population (74.16%) that expressed B lymphoid lineage. White Precursor Cell (WPC) channel test showed an abnormal lymphocytes population. The differential diagnosis of patients with dominant mature lymphocytes BSE with cytoplasmic projections was CLL and HCL. Immunophenotyping of CLL showed positive results on B cell markers (CD19, CD20, CD79a, and HLA-DR) with aberrant CD5. However, in such an HCL case like this, there were strongly positive results on B cell markers but the absence of aberrant CD5. This study was supported by the presence of abnormal lymphocytes population in the WPC test. The diagnosis of HCL in this patient was based on interpretation of BSE and immunophenotyping, supported by the WPC test.

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The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders

Blood ◽

10.1182/blood.v83.6.1558.bloodjournal8361558 ◽

1994 ◽

Vol 83 (6) ◽

pp. 1558-1562 ◽

Cited By ~ 1

Author(s):

E Matutes ◽

R Morilla ◽

K Owusu-Ankomah ◽

A Houlihan ◽

D Catovsky

Keyword(s):

Differential Diagnosis ◽

Monoclonal Antibodies ◽

B Cell ◽

Hairy Cell Leukemia ◽

Lymphocytic Leukemia ◽

Low Grade ◽

Hairy Cell ◽

Cell Leukemia ◽

Splenic Lymphoma ◽

Weak Surface

Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chain restriction (kappa/lambda: 1.5/1) with moderate to strong intensity of membrane Ig staining. Cells from most cases (> 80%) were CD24+, FMC7+, and expressed strongly membrane CD22. The monoclonal antibodies CD10, CD23, and CD38 were positive in one-third of the cases; CD11c in 47%; and CD25 in 25% of cases. A minority of cases (< 20%) were positive with HC2, B-ly-7, and CD5. However, none of the 19 CD5+ cases had the phenotype characteristic of chronic lymphocytic leukemia (CD5+, CD23+, FMC7-, weak surface Ig and membrane CD22). None of the 17 CD25+ cases had the immunophenotype typical of hairy cell leukemia (CD25+, CD11c+, HC2+, B-ly-7+). HC2 and B-ly-7 were the most useful reagents to distinguish SLVL from hairy cell leukemia. Our findings demonstrate that SLVL has a distinct immunologic profile and that monoclonal antibodies are important for the differential diagnosis between this disease and other B-lymphoproliferative disorders with which SLVL can be confused.

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The immunophenotype of splenic lymphoma with villous lymphocytes and its relevance to the differential diagnosis with other B-cell disorders

Blood ◽

10.1182/blood.v83.6.1558.1558 ◽

1994 ◽

Vol 83 (6) ◽

pp. 1558-1562 ◽

Cited By ~ 123

Author(s):

E Matutes ◽

R Morilla ◽

K Owusu-Ankomah ◽

A Houlihan ◽

D Catovsky

Keyword(s):

Differential Diagnosis ◽

Monoclonal Antibodies ◽

B Cell ◽

Hairy Cell Leukemia ◽

Lymphocytic Leukemia ◽

Low Grade ◽

Hairy Cell ◽

Cell Leukemia ◽

Splenic Lymphoma ◽

Weak Surface

Abstract Splenic lymphoma with villous lymphocytes (SLVL) is a low-grade disorder that regularly presents with peripheral blood involvement. We describe the immunophenotype of the circulating cells from 100 SLVL patients whose disease has been characterized on clinical, morphologic, and histologic grounds. Cells from all cases expressed B-cell antigens (CD19 and CD37) and/or HLA-Dr and showed light chain restriction (kappa/lambda: 1.5/1) with moderate to strong intensity of membrane Ig staining. Cells from most cases (> 80%) were CD24+, FMC7+, and expressed strongly membrane CD22. The monoclonal antibodies CD10, CD23, and CD38 were positive in one-third of the cases; CD11c in 47%; and CD25 in 25% of cases. A minority of cases (< 20%) were positive with HC2, B-ly-7, and CD5. However, none of the 19 CD5+ cases had the phenotype characteristic of chronic lymphocytic leukemia (CD5+, CD23+, FMC7-, weak surface Ig and membrane CD22). None of the 17 CD25+ cases had the immunophenotype typical of hairy cell leukemia (CD25+, CD11c+, HC2+, B-ly-7+). HC2 and B-ly-7 were the most useful reagents to distinguish SLVL from hairy cell leukemia. Our findings demonstrate that SLVL has a distinct immunologic profile and that monoclonal antibodies are important for the differential diagnosis between this disease and other B-lymphoproliferative disorders with which SLVL can be confused.

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Monoclonal antibodies reactive with hairy cell leukemia [see comments]

Blood ◽

10.1182/blood.v74.1.320.bloodjournal741320 ◽

1989 ◽

Vol 74 (1) ◽

pp. 320-325 ◽

Cited By ~ 1

Author(s):

L Visser ◽

A Shaw ◽

J Slupsky ◽

H Vos ◽

S Poppema

Keyword(s):

Monoclonal Antibodies ◽

B Cell ◽

Hairy Cell Leukemia ◽

Small Population ◽

Lymphocytic Leukemia ◽

Hairy Cell ◽

Cell Leukemia ◽

Hairy Cells ◽

A Minor ◽

Insight Into

Monoclonal antibodies reactive with hairy cell leukemia were developed to aid in the diagnosis of this subtype of B cell chronic lymphocytic leukemia and to gain better insight into the origin of hairy cells. Three antibodies were found to be of value in the diagnosis of hairy cell leukemia. Antibody B-ly 2 can be considered a pan-B cell reagent and generally reacts similar to CD22 antibodies. Antibody B-ly 6 is reactive with the same antigen as CD11c (p150/95), an antigen that is present on hairy cell leukemia, macrophages, and a minor subpopulation of lymphocytes. Antibody B-ly 7 is a unique antibody reactive with 144 Kd antigen present only on hairy cell leukemia and a very small population of normal B lymphocytes. This subpopulation may be the counterpart of hairy cells.

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B-ly-7, a monoclonal antibody reactive with hairy cell leukemia, also defines an activation antigen on normal CD8+ T cells [see comments]

Blood ◽

10.1182/blood.v76.5.959.959 ◽

1990 ◽

Vol 76 (5) ◽

pp. 959-964 ◽

Cited By ~ 26

Author(s):

SP Mulligan ◽

P Travade ◽

E Matutes ◽

C Dearden ◽

L Visser ◽

...

Keyword(s):

Monoclonal Antibody ◽

T Cells ◽

T Cell ◽

B Cell ◽

Cd8 T Cells ◽

Peripheral Blood ◽

Hairy Cell Leukemia ◽

Hairy Cell ◽

Cell Leukemia ◽

Activation Antigen

Abstract We undertook a study to determine the specificity of the monoclonal antibody, B-ly-7, for hairy cell leukemia (HCL) by examining the expression in 150 samples from B-cell lymphoproliferative diseases as well as screening for reactivity in a number of other hematologic malignancies. Within the B-cell lineage we found that the expression of B-ly-7 was highly specific for HCL and reacted with all 28 cases examined, as well as with 3 of 9 cases of a variant form of HCL. Cells of other closely related B-cell disorders, prolymphocytic leukemia, and splenic lymphoma with villous lymphocytes were negative. Investigation of the peripheral blood and bone marrow of patients with HCL before and after treatment with alpha-interferon or deoxycoformycin suggests that B-ly-7 may be useful in the assessment of minimal disease after therapy. In addition to HCL, we found that B-ly-7 was positive with cells of three mature, CD4+ T-cell malignancies. In view of the reactivity with malignancies of activated B and T cells, we searched for the expression of B-ly-7 on activated, normal B and T cells and found that B-ly-7 reacted specifically with activated normal peripheral blood CD8+ T cells. B-ly-7 has a number of applications, including the precise classification of mature B-cell neoplasia and the diagnosis HCL and its assessment after treatment. In addition, B-ly-7 recognizes a small subset of T-cell disorders. Its expression on these malignancies and on in vitro activated peripheral blood CD8+ T cells suggests that B- ly-7 detects a lymphocyte activation antigen.

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CD11c (LEU-M5) expression characterizes a B-cell chronic lymphoproliferative disorder with features of both chronic lymphocytic leukemia and hairy cell leukemia [see comments]

Blood ◽

10.1182/blood.v76.11.2360.bloodjournal76112360 ◽

1990 ◽

Vol 76 (11) ◽

pp. 2360-2367

Author(s):

CA Hanson ◽

TE Gribbin ◽

B Schnitzer ◽

JA Schlegelmilch ◽

BS Mitchell ◽

...

Keyword(s):

Chronic Lymphocytic Leukemia ◽

B Cell ◽

Hairy Cell Leukemia ◽

Lymphoproliferative Disorder ◽

Cellular Adhesion ◽

Lymphocytic Leukemia ◽

Tartrate Resistant Acid Phosphatase ◽

Hairy Cell ◽

Cell Leukemia ◽

Bone Marrow Biopsies

Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.

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CD11c (LEU-M5) expression characterizes a B-cell chronic lymphoproliferative disorder with features of both chronic lymphocytic leukemia and hairy cell leukemia [see comments]

Blood ◽

10.1182/blood.v76.11.2360.2360 ◽

1990 ◽

Vol 76 (11) ◽

pp. 2360-2367 ◽

Cited By ~ 39

Author(s):

CA Hanson ◽

TE Gribbin ◽

B Schnitzer ◽

JA Schlegelmilch ◽

BS Mitchell ◽

...

Keyword(s):

Chronic Lymphocytic Leukemia ◽

B Cell ◽

Hairy Cell Leukemia ◽

Lymphoproliferative Disorder ◽

Cellular Adhesion ◽

Lymphocytic Leukemia ◽

Tartrate Resistant Acid Phosphatase ◽

Hairy Cell ◽

Cell Leukemia ◽

Bone Marrow Biopsies

Abstract Chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HCL) are two common chronic lymphoproliferative disorders, each having characteristic clinical, morphologic, and immunologic features. Phenotypically, CD5 reactivity in CLL and CD11c (Leu-M5) reactivity in HCL have characterized these two leukemias among B-cell disorders. In this study, we report 14 cases of a novel chronic lymphoproliferative disorder characterized by lymphocytosis and CD11c expression, but morphologically similar to CLL. The patients' ages ranged from 46 to 81 years (median 62). Eleven had palpable splenomegaly, five with markedly enlarged spleens; only one patient had generalized lymphadenopathy. The white blood cell count ranged from 5.2 to 131.0 x 10(9)/L (median 20.8). The morphologic diagnosis in all cases was CLL, with the cells usually having abundant cytoplasm. No morphologic features, of hairy cells were evident; tartrate-resistant acid phosphatase cytochemistry was negative in all cases. Bone marrow biopsies were available in 8 of 14. Four showed focal nodular infiltrates and two had diffuse infiltrates similar to CLL; two showed only minimal interstitial involvement. All cases expressed multiple B-cell markers, and 12 of 14 had monoclonal surface immunoglobulin. The leukemic cells of all cases strongly expressed CD11c, while CD5 was expressed in 7 of 14; only 1 of the 14 cases expressed the lymph node homing receptor, Leu-8. This unique group of leukemias appears to represent the malignant transformation of lymphocytes arising from a stage of lymphocyte differentiation between that found in typical cases of CLL and that of HCL. CD11c is known to have an important function in cellular adhesion and may be important in determining the pattern of lymphocyte tissue distribution found in this group of patients.

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