CD56+/CD4+ lymphomas and leukemias are morphologically, immunophenotypically, cytogenetically, and clinically diverse.
CD56, a neural adhesion molecule, is a marker of natural killer (NK) lymphocytes as well as a subgroup of CD8+ T cells. Normal lymphocytes with a CD56/CD4 phenotype are scarce. Physiologic increases may occur in patients with immunosuppression, chronic inflammation, and autoimmune disorders. We report 4 cases of lymphomas/leukemias with the unusual CD56/CD4 phenotype. Two were of T-cell and 2 of true NK-cell origin. The T-cell lymphomas had large granular lymphocyte morphologic features and splenomegaly. One patients had a benign course; the other died within months of the leukemia diagnosis. The 2 NK cell lymphomas had blastic morphologic features, initially involved skin, and had a very aggressive clinical course; 1 patient died of acute leukemia, and 1 had recurrence after bone marrow transplantation. Cytogenetic analyses did not show a consistent pattern of abnormalities. The NK lymphoma with acute leukemia had a t(2;5) but was CD30- and anaplastic lymphoma kinase negative. Although CD56+/CD4+ lymphomas/leukemias are a heterogeneous group, there may be a distinct subgroup of NK lymphoblastoid lymphomas of the skin, judging from our cases, as well as those previously reported.
Rakozy
C K
CK
Department of Pathology, Wayne State University School of Medicine and Karmanos Cancer Center, Detroit, MI, USA.
Mohamed
A N
AN
Vo
T D
TD
Khatib
G
G
Long
P M
PM
Eilender
D
D
Palutke
M
M
D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D015704:CD4 Antigens / Q000032:analysis*; D019002:CD56 Antigen / Q000032:analysis*; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000276:immunology / Q000473:pathology; D007938:Leukemia / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008223:Lymphoma / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D014066:Palatine Tonsil / Q000473:pathology; D012867:Skin / Q000473:pathology; D013154:Spleen / Q000473:pathology
D015704:CD4 Antigens; D019002:CD56 Antigen