“The Great Mimicker”: An Unusual Etiology of Cytopenia, Diffuse Lymphadenopathy, and Massive Splenomegaly

Abstract

Sarcoidosis is an idiopathic multisystem disease characterized by the formation of noncaseating granulomas. It frequently presents with pulmonary infiltrates and bilateral hilar and mediastinal lymphadenopathy. Splenic involvement is common, but massive splenomegaly is a rare occurrence. Sarcoidosis is known as “the great mimicker” (or “the great imitator”) since it exhibits a myriad of symptoms, mimicking other inflammatory, infectious, and neoplastic conditions, including lymphoma. Herein, we report the case of a 44-year-old male patient who was found to have bicytopenia, hypercalcemia, diffuse lymphadenopathy, and massive splenomegaly, a constellation of findings suggestive of underlying lymphoma. Interestingly, lymph node biopsy showed noncaseating granulomas suggestive of sarcoidosis, without evidence of malignancy.

Lymphadenopathy: Differentiation between Tuberculosis and Other Non-Tuberculosis Causes like Follicular Lymphoma

“…Abdominal USG is an important non-invasive investigation in suspicious cases, since 50% of FL patients may have splenomegaly ( 26 ). Especially, if splenomegaly is massive, some of the differential diagnosis are chronic lymphocytic leukemia, non-Hodgkin lymphoma, chronic myelocytic leukemia, polycythemia vera, myelofibrosis with myeloid metaplasia, or hairy cell leukemia ( 27 , 28 ). Splenic involvement in TB usually occurs in the military form and is very rare ( 29 , 30 ).…”

Massive Splenomegaly Indicating Sarcoidosis

“…1,2 CT scan features can show a homogeneous spleen, but also visible nodules with heterogeneous features due to coalescence of sarcoid granuloma. 3,4 Most authors consider that massive splenomegaly is defined when it extends into the left lower quadrant or pelvis, or when it has crossed the midline of the abdomen, but others consider that it is characterized by a weight over 1000-1500 g or a largest dimension >20 cm. 2 In a review including 6074 cases with sarcoidosis, massive splenomegaly was described in 20 patients (3%).…”

“…4 Differential diagnosis includes hematologic malignancies (myeloproliferative syndrome and lymphoma), infections (visceral leishmaniasis and hyperreactive malarial splenomegaly syndrome), and infiltrative conditions (Gaucher disease). 3,4 No treatment is required when the splenomegaly is asymptomatic. When it is disabling, treatment relies on oral corticosteroids, which were highly effective in our patient.…”

A Case of Sarcoidosis Mimicking Lymphoma Confounded by Cognitive Decline

“…While the underlying cause of sarcoidosis is not well understood, the immune system plays a central role in its pathogenesis. Noncaseating granulomas are seen histologically, which are also associated with other pathologies such as malignancy, infection (mycobacteria, fungi, parasites, histoplasmosis), and occupational and environmental exposures (such as beryllium or silica) [ 3 ]. It is important to first rule out these disease processes before making the diagnosis of sarcoidosis.…”