Primary Anaplastic Lymphoma Kinase–Negative Anaplastic Large Cell Lymphoma of the Brain in a Patient With Acquired Immunodeficiency Syndrome

Abstract

Anaplastic large cell lymphoma is a unique diagnostic subcategory of the T-cell lymphomas in the current World Health Organization classification. Representing approximately 3% of adult and 10% to 30% of childhood non-Hodgkin lymphomas, anaplastic large cell lymphoma classically consists of CD30+ large lymphoid cells with abundant cytoplasm and pleomorphic, often horseshoe-shaped or kidney-shaped nuclei. Among the reported nodal and extranodal sites of occurrence, the gastrointestinal tract and central nervous system have rarely been noted. We report a case of primary anaplastic lymphoma kinase–negative anaplastic large cell lymphoma in the brain of a 46-year-old patient with acquired immunodeficiency syndrome. T-cell lineage was confirmed by T-cell receptor γ chain gene rearrangements using polymerase chain reaction, and extra copies of the anaplastic lymphoma kinase gene of chromosome 2 were demonstrated by fluorescence in situ hybridization analysis. To our knowledge, primary anaplastic large cell lymphoma of the brain has not previously been reported in acquired immunodeficiency syndrome.

Clinical Features and Prognostic Factors for Primary Anaplastic Large Cell Lymphoma of the Central Nervous System: A Systematic Review

“…Approximately 19% of PCNSL patients have HIV in the United States [46]. On the other hand, for primary ALCL of the CNS, only one case was reported to be associated with AIDS [38]; all other cases were considered immunocompetent.…”

Anaplastic large cell lymphoma in human immunodeficiency virus‐infected people and solid organ transplant recipients

“…ALCL has rarely been described in immunosuppressed people in individual case reports or case series 6–13,15–17,19–23 . In general, T‐cell lymphomas are less common than B‐cell lymphomas in HIV‐infected people and transplant recipients 1,2 .…”

“…ALCL has rarely been described in immunosuppressed people in individual case reports or case series. [6][7][8][9][10][11][12][13][15][16][17][19][20][21][22][23] In general, T-cell lymphomas are less common than B-cell lymphomas in HIV-infected people and transplant recipients. 1,2 Also, in the absence of compelling data, ALCL has not been considered an HIV-associated lymphoma like DLBCL or Burkitt lymphoma, which makes our findings intriguing.…”

“…4,5 Although most NHLs occurring in the immunosuppressed populations are B-cell malignancies, two prior studies reported markedly elevated risk of systemic anaplastic large cell lymphoma (ALCL) among HIV-infected people and transplant recipients. 1,2 Several case reports and series have described tumour characteristics of ALCLs arising among HIV-infected people [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] and transplant recipients. [21][22][23] ALCL is a rare T-cell lineage lymphoma that comprises approximately 2Á4% of all NHLs and 16% of all peripheral T-cell lymphomas in adults.…”

Central nervous system ALK-negative anaplastic large cell lymphoma with IRF4/DUSP22 rearrangement

“…ALCLs involving the CNS are very rare, have been ALK-positive, ALK-negative or with unknown ALK status, and tend to exhibit aggressive behavior [5,13,14]. Only 15 cases of primary CNS ALK-negative ALCLs have been reported [5,13,[15][16][17][18][19][20][21][22][23][24].…”