PMC7521157_01
Axillary lymph node biopsy (April 2017; A-D) and gastric biopsy (February 2019; E-G). (A) High power view of the lymph node shows a few large LP cells scattered within a background of small lymphocytes and histiocytes. (B) CD20 immunostaining in a pattern A (classical) nodule highlighting many small B cells and scattered large LP cells (red circles). (C) CD20 immunostaining in pattern C [nodular with many extranodular lymphocyte predominant (LP) cells] showing the edge of a nodule (asterisk), and scattered large LP cells (red circles) outside of the nodule (D) CD20 immunostaining showing large CD20+ atypical cells (red circles) arranged in large nodules with essentially no small B cells in the background (pattern D: T-cell-rich nodular). (E) Low-power view of the gastric biopsies showing a dense and destructive cellular infiltrate. (F) The infiltrate comprises lymphoid cells including large atypical elements. (G) CD79a highlights the rather numerous large atypical B cells.
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F1
PET-CT showed enlargement of multiple peripheral, thoracic, and abdominal lymph nodes, several hypermetabolic foci in the liver, splenomegaly and focal pancreatic uptake [maximal standardized uptake value (SUV) 23.8, at hepatic hilum], and an axillary lymph node biopsy (Figures 1A-D) showed NLPHL comprising different immunoarchitectural patterns, mostly C [nodular with many extranodular lymphocyte predominant (LP) cells] and D (T-cell-rich nodular), with a minor component of classical A pattern (B-cell rich nodular). A bulging of the gastric wall was observed through gastroscopy, and biopsies (Figures 1E-G) revealed an atypical destructive lymphoid infiltrate in the mucosa, containing a moderate number of large CD79a+, BCL2+, CD10-lymphoid cells, surrounded by abundant T cells, interpreted as relapse with THRLBCL-like lymphoma.
Selected axial T1 weighted images from brain MRI with contrast (A,B). (A) Diffuse gyriform leptomeningeal enhancement on both cerebral hemispheres. (B) Enhancement of the cisternal portion of the right 6th cranial nerve (arrow).
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F2
The initial work-up showed intracranial hypertension (up to 50 cm H2O), lymphocytic meningitis (pleocytosis up to 220 cells/mul, between 80 and 95% lymphocytes), elevated cerebrospinal fluid (CSF) lactate levels (up to 9 mmol/l), very low CSF-to-serum glucose levels (ratio down to 0.12), increased CSF protein (up to 12 g/l), as well as diffuse leptomeningeal and cranial nerve enhancement, without any parenchymal lesion on magnetic resonance imaging (Figure 2).
Brain biopsy (A-E) and CSF (F). (A) Low power view of the brain cortical biopsy showing cellular deposits on the meningeal surface. (B) The cellular foci comprise many large atypical cells. (C) The latter are positive for CD79a. (D) CD3 stains many admixed small T cells. (E) Ki67 stains the nuclei of most large cells. (F) CSF contains a few large atypical cells and many small lymphocytes.
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F3
Brain autopsy. (A) Low power view of leptomeningeal spaces enlarged by a dense cellular infiltrate. (B) The infiltrate comprises scattered large lymphoid cells (arrows), numerous small lymphocytes, and histiocytes. (C) CD79a highlights the large atypical B cells (arrows). (D) CD68 underscores the histiocyte-rich background.
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F4
THRLBCL-like characteristics (Figure 4). The infiltrate consisted of small CD3+ lymphocytes, histiocytes, and scattered large hyperchromatic lymphoid cells that were positive for CD79a and CD19.