UNUSUAL PRESENTATION OF HAIRY CELL LEUKEMIA: A CASE REPORT

Hairy cell leukemia is a chronic B cell lymphoproliferative disorder,which is uncommon and constitutes around two percent of hematolymphoid malignancies. HCL commonly involves bone marrow and spleen and rarely peripheral blood . Splenomegaly is a prominent feature and is seen in around 70 to 100% of HCL cases as reported in various case reports. Sometimes the absence of splenomegaly rules out the diagnosis of HCL and is misdiagnosed as aplastic anemia. Thus the aim of our study is to understand the importance and keep a high level of suspicion in such cases. As in present case there was no evidence of splenomegaly clinically or radiology, but the morphologic features on biopsy had suggested HCL which was further confirmed on immunophenotyping. The purpose of this case report is to highlight the importance of the fact that HCL can present even without splenomegaly. Keywords: Hairy cell leukemia, Splenomegaly, Immunohistochemistry

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CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen

Current Oncology ◽

10.3390/curroncol28060430 ◽

2021 ◽

Vol 28 (6) ◽

pp. 5124-5147

Author(s):

John J. Schmieg ◽

Jeannie M. Muir ◽

Nadine S. Aguilera ◽

Aaron Auerbach

Keyword(s):

Bone Marrow ◽

B Cell ◽

Hairy Cell Leukemia ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Lymphoproliferative Disorders ◽

Low Grade ◽

Hairy Cell ◽

Cell Leukemia ◽

Red Pulp

CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.

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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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VILLOUS LYMPHOCYTES IN BLOOD AND BONE MARROW IN SOME FORMS OF B-CELL LYMPHOID MALIGNANCIES

EUREKA Life Sciences ◽

10.21303/2504-5695.2020.001429 ◽

2020 ◽

pp. 29-33

Author(s):

Alyona Polishchuk ◽

Michael Zavelevich ◽

Daniil Gluzman

Keyword(s):

Bone Marrow ◽

B Cell ◽

Hairy Cell Leukemia ◽

Marginal Zone ◽

Marginal Zone Lymphoma ◽

Splenic Marginal Zone Lymphoma ◽

Hairy Cell ◽

Cell Leukemia ◽

Trap Activity ◽

Red Pulp

The cytological and immunocytochemical features of the lymphocytes with villous morphology in peripheral blood and bone marrow in some B-lymphoproliferative disorders were studied. The diagnosis of hairy cell leukemia, a hairy cell leukemia variant, splenic marginal zone lymphoma and splenic diffuse red pulp small B-cell lymphoma was ascertained in accordance with the new revision of the WHO classification (2016). The neoplastic cells of hairy cell leukemia were determined by the presence of high tartrate resistant acid phosphatase (TRAP) activity. Cell surface expression of CD19, CD20 and CD21 antigens was detected. Also, the expression of CD25, CD103 and CD200, and in some cases cyclin D1, was found out. CD5, CD10 and CD23 were not detected. The immunophenotype of cells in splenic marginal zone lymphoma with villous processes also corresponded to the mature B cells. The expression of CD19, CD20 and CD21 was observed in all cases, CD11c – in 50% of patients, CD25 or CD5 – in 10% of patients. In 80% of patients, the pathologic cells did not show TRAP activity. In the bone marrow and peripheral blood cells of patients with diffuse red pulp lymphoma, TRAP activity was not detected. An immunophenotype in the hairy cell leukemia variant was different from those of classic HCL (CD19+CD20+CD22+CD103+CD11c+CD5–CD10–CD23–). Characterized immunophenotypical markers, which have differential diagnostic values in several forms of lymphoid tumors of B cell origin, will be important for the choice of treatment methods and prognosis

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Molecular and flow cytometry characterization during the follow-up of three simultaneous lymphoproliferative disorders: Hairy cell leukemia, monoclonal B-cell lymphocytosis, and CD4++/CD8+/−dim T-large granular lymphocytosis-A case report

Cytometry Part B Clinical Cytometry ◽

10.1002/cyto.b.20579 ◽

2010 ◽

Vol 80B (3) ◽

pp. 195-200 ◽

Cited By ~ 5

Author(s):

P. Garrido ◽

P. Jiménez ◽

C. Sánchez ◽

F. Valero ◽

A. Balanzategui ◽

...

Keyword(s):

Flow Cytometry ◽

Case Report ◽

B Cell ◽

Hairy Cell Leukemia ◽

Lymphoproliferative Disorders ◽

Hairy Cell ◽

Cell Leukemia

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A Case Report of Hairy Cell Leukemia Presenting Concomitantly with Sweet Syndrome

Case Reports in Medicine ◽

10.1155/2014/823286 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 5

Author(s):

Mohammad Alkayem ◽

Waina Cheng

Keyword(s):

Bone Marrow ◽

Case Report ◽

Skin Biopsy ◽

Bone Marrow Biopsy ◽

Hairy Cell Leukemia ◽

Hairy Cell ◽

Cell Leukemia ◽

Sweet Syndrome ◽

Purine Analogs

Hairy cell leukemia and Sweet syndrome are both uncommon hematological diagnoses. We present a patient who was admitted with fevers, pancytopenia, pneumonia, and rash. Diagnostic bone marrow biopsy demonstrates Hairy cell Leukemia and skin biopsy demonstrates neutrophils infiltration consistent with Sweet syndrome. The patient was treated with purine analogs with resolution of the cytopenias, infection, and rash.

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A Case of CD5 Positive Hairy Cell Leukemia withBRAF V600EMutation and Lymph Node Involvement; Unique Case Report and Review of the Literature:

Blood ◽

10.1182/blood-2020-140039 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 17-18

Author(s):

Sara Javidiparsijani ◽

Yun Kyong Choi ◽

Arnaldo A. Arbini

Keyword(s):

Bone Marrow ◽

Lymph Node ◽

B Cell ◽

Hairy Cell Leukemia ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Lymph Node Involvement ◽

Hairy Cell ◽

Cell Leukemia ◽

Node Involvement

Objective: Hairy cell leukemia (HCL) is distinct indolent neoplasm of small mature B cells accounting for 2% of the lymphoid leukemias. The classic immunophenotypic profile of HCL includes bright expression of B cell surface antigen and immunoglobulins and lack of both CD5 and CD10. The tumor cells are predominantly present in the spleen, peripheral blood and bone marrow. Aberrant expression of CD5 and lymph node involvement are very rare. A case of CD5(+) HCL with a documentedBRAF V600Emutation has not been previously reported. This case is also unique due to lymph node involvement by HCL. To our knowledge, this is the only case report of a documentedBRAF V600Emutated CD5(+) HCL along with lymph node involvement in the literature. Method: We are presenting a 45 years old man with multiple prior medical conditions including diabetes, COPD and interstitial pneomonitis with new onset of B-symptoms and neutropenia/monocytopenia. Peripheral blood flow cytometry revealed 21% CD5(+) CD10(-) monoclonal B cell population. Further workup revealed the neoplastic cells are positive for CD11c, CD103 and CD25. Bone marrow study revealed an extensive involvement of the marrow.By immunohistochemistry, the cells are also positive for CD5, CD20, BRAF and cyclin D1. Cytogenetic studies show that the cells are negative t(11;14) and molecular studies revealedBRAF V600Emutation. Reexamination of the prior wedge lung resection revealed intrathoracic lymph node involvement. Results and conclusion: The most common differential diagnoses of a CD5(+) B cell lymphoma involving the bone marrow is chronic lymphocytic leukemia (CLL) and mantle cell lymphoma. The key into the diagnosis of this case was marked monocytopenia that lead to addition of HCL antibody tubes despite the CD5 positivity and detection of CD25, CD103 and CD11c antigens in the leukemic cells. Bone marrow biopsy show extensive involvement by a CD5(+) cyclin D1(+) B cell lymphoma. The most important differential diagnosis in this case was mantle cell lymphoma. The diagnosis of HCL was confirmed by detection ofBRAF V600Emutation and absence of t (11;14). Retrospective examination of the intrathoracic lymph nodes show one lymph node involvement by hairy cell leukemia cells. The patient was treated with Vemurafenib and his follow up flow cytometry revealed only 0.60% leukemic involvement. Incorporation of clinical data at the time of flow cytometric examination is essential to aid the hematopathologist in having a broader differential diagnoses and addition of appropriate tests that lead to the more accurate diagnosis and treatment. Disclosures No relevant conflicts of interest to declare.

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