Clinicopathologic Features of Peripheral T-Cell Lymphoma in Sub-Saharan Africa

Abstract

Objectives: Peripheral T-cell lymphomas (PTCLs) are heterogeneous, clinically aggressive, and rare. Subtype distribution varies by geographic location; however, data from sub-Saharan Africa (SSA) are lacking. We sought to elucidate clinicopathologic features of PTCL in SSA.

Methods: We reviewed PTCL consultation cases from three SSA countries. PTCL subtype was determined per 2017 World Health Organization classification. Cases with sufficient material were evaluated by polymerase chain reaction for human T-cell leukemia virus type 1 (HTLV-1) and T-cell receptor γ (TCRG) rearrangement.

Results: Among 32 cases, median age was 45 years and male-to-female ratio was 1.7. Thirty (94%) of 32 cases required additional workup for subclassification. PTCL, not otherwise specified (PTCL-NOS) was the most common subtype (13/32, 41%), followed by PTCL with T-follicular helper phenotype (6/32, 19%) and systemic anaplastic large cell lymphoma (6/32, 19%). Four (16%) of 25 cases were Epstein-Barr virus positive (EBV+) (2/2 extranodal natural killer/T-cell lymphoma, 1/13 PTCL-NOS, and 1/4 angioimmunoblastic T-cell lymphoma with EBV+ immunoblasts). Two (15%) of 13 patients with PTCL-NOS were human immunodeficiency virus positive. No cases with evaluable DNA (0/15) were HTLV-1 positive, and 9 of 10 showed clonal TCRG rearrangements.

Conclusions: In comparison to Western studies, PTCLs from SSA show similar subtype distribution and male predominance but a younger age at diagnosis. Appropriate diagnosis of PTCL requires extensive ancillary testing not readily available in low-income countries, including much of SSA.

Keywords: Epstein-Barr virus; Low/middle-income countries; Peripheral T-cell lymphoma; Sub-Saharan Africa.

Image 1

Immunophenotypic characterization of peripheral T-cell lymphoma (PTCL). A, PTCL not otherwise specified (H&E, ×400) showing a dense CD4-positive atypical T-cell infiltrate (B, ×400) with loss of CD7 (C, ×400). D, Angioimmunoblastic T-cell lymphoma demonstrating characteristic patent subcapsular sinus (H&E, ×200). PD1 expression (E, ×200) and follicular dendritic cell hyperplasia highlighted by CD21 (F, ×100). G, Systemic anaplastic large cell lymphoma demonstrating characteristic hallmark cells (H&E, ×200 and ×1,000 [inset]) and strong and diffuse staining for ALK (H, ×200) and CD30 (I, ×200).

Image 1

Immunophenotypic characterization of peripheral T-cell lymphoma (PTCL). A, PTCL not otherwise specified (H&E, ×400) showing a dense CD4-positive atypical T-cell infiltrate (B, ×400) with loss of CD7 (C, ×400). D, Angioimmunoblastic T-cell lymphoma demonstrating characteristic patent subcapsular sinus (H&E, ×200). PD1 expression (E, ×200) and follicular dendritic cell hyperplasia highlighted by CD21 (F, ×100). G, Systemic anaplastic large cell lymphoma demonstrating characteristic hallmark cells (H&E, ×200 and ×1,000 [inset]) and strong and diffuse staining for ALK (H, ×200) and CD30 (I, ×200).

Image 2

Immunophenotypic characterization of primary cutaneous peripheral T-cell lymphoma. A, Folliculotropic mycosis fungoides (H&E, ×100) showing an atypical CD4-positive T-cell infiltrate (B, ×100) with scattered CD30-positive large cells (C, ×400). D, E, Primary cutaneous anaplastic large cell lymphoma with an atypical T-cell infiltrate extending to the deep dermis (H&E, ×40 and ×1,000) and adjacent granulomatous inflammation with giant cells (F, H&E, ×200).

Image 2

Immunophenotypic characterization of primary cutaneous peripheral T-cell lymphoma. A, Folliculotropic mycosis fungoides (H&E, ×100) showing an atypical CD4-positive T-cell infiltrate (B, ×100) with scattered CD30-positive large cells (C, ×400). D, E, Primary cutaneous anaplastic large cell lymphoma with an atypical T-cell infiltrate extending to the deep dermis (H&E, ×40 and ×1,000) and adjacent granulomatous inflammation with giant cells (F, H&E, ×200).

Image 3

Immunophenotypic characterization extranodal natural killer/T-cell lymphoma (ENKTL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). A, ENKTL (H&E, ×400) demonstrating positivity for CD56 (B, ×400) and Epstein-Barr virus by Epstein-Barr virus–encoded RNA in situ hybridization (C, ×400). D, MEITL involving intestinal mucosa (H&E, ×100) showing an atypical T-cell infiltrate positive for CD56 (E, ×100) and CD3 (F, ×100).

Image 3

Immunophenotypic characterization extranodal natural killer/T-cell lymphoma (ENKTL) and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). A, ENKTL (H&E, ×400) demonstrating positivity for CD56 (B, ×400) and Epstein-Barr virus by Epstein-Barr virus–encoded RNA in situ hybridization (C, ×400). D, MEITL involving intestinal mucosa (H&E, ×100) showing an atypical T-cell infiltrate positive for CD56 (E, ×100) and CD3 (F, ×100).

Image 4

Immunophenotypic characterization of hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (T-LGLL). A, HSTL demonstrating monotonous lymphocytes involving splenic cords and sinuses (H&E, ×200). B, Neoplastic lymphocytes are positive for CD3 (×400) and T-cell receptor δ (C, ×400). D, T-LGLL showing a left-shifted mildly hypercellular marrow (H&E, ×200) and occasional large granular lymphocytes in the peripheral blood (inset, ×1,000). Neoplastic lymphocytes showed positivity for CD57 (E, ×400) and granzyme B (F, ×400).

Image 4

Immunophenotypic characterization of hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (T-LGLL). A, HSTL demonstrating monotonous lymphocytes involving splenic cords and sinuses (H&E, ×200). B, Neoplastic lymphocytes are positive for CD3 (×400) and T-cell receptor δ (C, ×400). D, T-LGLL showing a left-shifted mildly hypercellular marrow (H&E, ×200) and occasional large granular lymphocytes in the peripheral blood (inset, ×1,000). Neoplastic lymphocytes showed positivity for CD57 (E, ×400) and granzyme B (F, ×400).