Korean J Hematol.  2009 Dec;44(4):255-260. 10.5045/kjh.2009.44.4.255.

A Case of CD5 Negative Chronic Lymphocytic Leukemia

• Hwang K
• Park CJ
• Park SJ
• Jang S
• Chi HS
• Cha CH
• Huh JR
• Suh CW

• KMID: 2252105
• DOI: http://doi.org/10.5045/kjh.2009.44.4.255

Abstract

Neoplastic lymphoid cells of chronic lymphocytic leukemia (CLL) typically co-express CD5 and CD23. CD5-negative CLL is a rare variant of CLL; only 1 case of it has been reported in Korea. We describe a case of CD5-negative CLL. A 48-year-old female complained of a palpable neck mass that had been present for over 1 year. The initial WBC count was 7,300/microliter, with 69% lymphocytes. A CT scan revealed multiple enlarged lymph nodes, both of each in the neck, axilla, and common iliac areas. The athologic results of the cervical lymph node was consistent with small lymphocytic lymphoma, of which tumor cells do not express CD5. In a bone marrow study, neoplastic lymphoid cells comprise 34.8% of all nucleated cells, which showed small size, round nuclei with clumped chromatin, and sparse cytoplasm. Immunophenotyping of small lymphoid cells displayed phenotypes that were CD45-, CD23-, CD20-, and CD19-positive, but CD5-negative. The patient was diagnosed with CD5-negative CLL, and has been followed up for 2.5 years after chemotherapy.

Keyword

Chronic lymphocytic leukemia; CD5 antigen; Immunophenotyping

MeSH Terms

Antigens, CD5
Axilla
Bone Marrow
Chromatin
Cytoplasm
Female
Humans
Immunophenotyping
Korea
Leukemia, Lymphocytic, Chronic, B-Cell
Lymph Nodes
Lymphocytes
Middle Aged
Neck
Phenotype
Antigens, CD5
Chromatin

Figure

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  Fig. 1. Cervical lymph node biopsy findings. (A) Diffuse infiltration of small lymphocytes in cervical lymph node (H&E stain, ×400), (B) Negative expression of CD5 on neoplastic lymphoid cells, (C) Positive expression of CD23 on neoplastic lymphoid cells, (D) Weak positive expression of CD20 on neoplastic lymphoid cells (B, C, D: immunohistochemistry on lymph node biopsy section, ×400).

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  Fig. 2. Peripheral blood, bone marrow aspirate and bone marrow biopsy findings. (A) Small lymphocyte in peripheral blood (Wright stain, ×1,000), (B) Frequent small lymphocytes in bone marrow aspirate (Wright stain, ×1,000), Nodular or interstitial infiltration of small lymphocytes in bone marrow clot section (C) and bone marrow biopsy (D) (H&E stain, ×400).

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  Fig. 3. Immunophenotyping of bone marrow aspirate. (A) Gating of nucleated cells expressing bright CD45 and low SSC, (B) Gated cells are small mononucleated cells, (C) Gated cells show CD23 positivity and CD5 negativity.

Reference

References

1. Sheikh SS, Kallakury BV, Al-Kuraya KA, Meck J, Hartmann DP, Bagg A. CD5-negative, CD10-negative small B-cell leukemia: variant of chronic lymphocytic leukemia or a distinct entity? Am J Hematol. 2002; 71:306–10.

2. Kim SI, Cho HI, Park MH. Leukemia and leukemia-related disorders in Korean. Korean J Hematol. 1998; 23:313–33.

3. Hallek M, Cheson BD, Catovsky D, et al. Guidelines for the diagnosis and treatment of chronic lymphocytic leukemia: a report from the international workshop on chronic lymphocytic leukemia updating the national cancer institute-working group 1996 guidelines. Blood. 2008; 111:5446–56.

4. World Health Organization classification of tumours of haematopoietic and lymphoid tissues. Muller-Hermelink HK, Montserrat E, Catovsky D, editors. Chronic lymphocytic leukemia/small lymphocytic lymphoma. 4th ed.IARC, USA: IARC press;2006. p. 180–2.

5. Okamoto M, Nakano S, Namura K, et al. CD5-negative chronic lymphocytic leukemia with indolent clinical course and autoimmune thrombocytopenia, successfully treated with rituximab. Am J Hematol. 2004; 77:413–5.

6. Huang JC, Finn WG, Goolsby CL, Variakojis D, Peterson LC. CD5-small B-cell leukemias are rarely classifiable as chronic lymphocytic leukemia. Am J Clin Pathol. 1999; 111:123–30.

7. Kim OK, Cheon JS, Kim HJ, et al. A case of CD5 Negative B-Cell chronic lymphocytic leukemia. Korean J Med. 1999; 56:235–9.

8. Cartron G, Linassier C, Bremond JL, et al. CD5 negative B-cell chronic lymphocytic leukemia: clinical and biological features of 42 cases. Leuk Lymphoma. 1998; 31:209–16.

9. Cavalcanti Junior GB, Sales VS, Cavalcanti e Silva DG, et al. Detection of CD5 in B-cell chronic lymphoproliferative diseases by flow cytometry: a strong expression in B-cell chronic lymphocytic leukemia. Acta Cir Bras. 2005; 20(Suppl 1):101–7.

10. Matutes E, Owusu-Ankomah K, Morilla R, et al. The immunological profile of B-cell disorders and proposal of a scoring system for the diagnosis of CLL. Leukemia. 1994; 8:1640–5.

11. Wang C, Amato D, Fernandes B. CD5-negative phenotype of monoclonal B-lymphocytosis of undetermined significance (MLUS). Am J Hematol. 2002; 69:147–9.

12. Yamada T, Ninomiya H, Horiuchi T, et al. CD5 negative B-cell chronic lymphocytic leukemia (B-CLL) associated with massive splenomegaly and without lymphadenopathy. Rinsho Ketsueki. 1995; 36:1230–2.

13. Keung YK, Buss D, Pettenati M, Powell BL. CD5-negative chronic lymphocytic leukemia or monoclonal B-lymphocytosis of undetermined significance? Am J Hematol. 2002; 70:334.

14. Specchia G, Albano F, Anelli L, et al. Concomitant tetrasomy 3q and trisomy 18 in CD5 (−), CD13 (+) chronic lymphocytic leukemia. Cancer Genet Cytogenet. 2002; 133:160–3.