Sarcoidosis-lymphoma syndrome: a diagnostic dilemma

Abstract

Sarcoidosis and lymphoma are generally thought of as being two mutually exclusive diseases that need to be considered in the differential diagnosis of patients with hilar/mediastianal lymphadenopathy. However, there are rare patients in whom both of these diseases coexist. These patients constitute a diagnostic challenge because their presentation (ie, clinical symptoms, imaging abnormalities and even pathology) may all be atypical when each individual disease is considered separately. In this report, we describe a patient who presented with such atypical features and was eventually diagnosed as having both sarcoidosis and a B-cell lymphoma with features of splenic marginal zone lymphoma (SMZL) simultaneously. To our knowledge, this is only the second reported case of SMZL and sarcoidosis in the same patient.

Keywords: cancer; cancer intervention; immunology; pathology; respiratory system.

Figure 1

Representative CT cuts of the thorax. (A) Contrast-enhanced cut demonstrating a 33×20 mm heterogenously enhancing subcarinal (white arrowheads) and bilateral 16 mm right and 11 mm left interlobar nodes (white arrows). (B) Lung window cut demonstrating bilateral patchy consolidation with basal predominance; the air space disease is a mixture of more nodular foci and larger more confluent areas of consolidation (*).

Figure 2

Lung, 100×, H&E: Lung parenchyma with well-formed non-caseating granulomata and conspicuous multinucleated giant cells.

Figure 3

Bone marrow aspirate, 1000× oil, Wright-Giemsa: The bone marrow aspirate shows increased numbers of plasma cells. Immunohistochemical studies performed on the bone marrow core biopsy (not shown) showed approximately 10%–15% plasma cells with cytoplasmic light chain restriction.

Figure 4

Gross image of intact spleen showing marked splenomegaly (3100 g, 30 cm in maximum dimension) with two enlarged perihilar lymph nodes below.

Figure 5

Splenic hiliar lymph node 100×, H&E: Marginal zone lymphoma associated with effacement of the normal lymph node architecture by a nodular infiltrate of small lymphocytes and plasma cells associated with disruption of follicular dendritic cell meshworks as noted by CD21 staining (inset, immunohistochemistry, 100×).

Figure 6

Spleen 400×, H&E: Marginal zone lymphoma with plasmacytic differentiation involving splenic parenchyma characterised by dense sheet-like proliferation of mature plasma cells with cytoplasmic lambda light chain expression (inset, lambda immunohistochemistry, 400×). Only rare plasma cells labelled with kappa (not shown).

Figure 7

Spleen 100×, H&E: spleen with non-caseating granulomata.