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.
Sarcoidosis is a chronic inflammatory disorder of unknown origin which occurs mainly in young people [
The symptoms of sarcoidosis, if present, are nonspecific. The presence of noncaseating granulomas is also not pathognomonic of the disease, as it can be seen in malignancy [
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. Surprisingly, lymph node biopsy showed noncaseating granulomas suggestive of sarcoidosis, without evidence of malignancy.
We report the case of a 44-year-old Caucasian male who was referred to our hospital by his primary physician for abnormal outpatient laboratory test values. The patient had been healthy until 5 months prior to admission, when he started to have progressively worsening fatigue. Outpatient blood tests revealed kidney injury, hypercalcemia, and anemia, findings that required hospitalization.
On the day of admission, the patient’s only complaint was severe fatigue. Upon further questioning, he admitted having a 70-pound unintentional weight loss over the last 18 months. He denied any fever, chills, night sweats, cough, rash, or joint or abdominal pain. His prior medical history consisted of diabetes mellitus, gout, and hyperlipidemia. The patient was a nonsmoker and had no allergies. His family history was noncontributory.
On physical exam, the patient’s body temperature was 98.6°F, blood pressure was 159/92 mmHg, and heart rate was 100/min. Cardiovascular and pulmonary exams were unremarkable. Left upper quadrant tenderness was noted on the abdominal exam, as well as a firm and enlarged spleen, which was palpable below the umbilicus. No rash, cervical, or axillary lymphadenopathy was identified.
Laboratory analysis showed a normocytic anemia with a hemoglobin of 6.7 g/dL, a hematocrit of 21.4%, and a mean corpuscular volume (MCV) of 82.3
Massive splenomegaly. (a) Sagittal sonographic view of the spleen showing a markedly enlarged and diffuse heterogeneous spleen (blue arrow) measuring 30 cm in length. (b) Coronal noncontrast CT of the abdomen and pelvis showing enlarged spleen reaching 33.6 cm in length.
Diffuse lymphadenopathy. (a) Transverse CT of the abdomen showing enlarged para-aortic lymph nodes (blue arrows) reaching 14 mm in the shortest axis. (b) Transverse CT of the pelvis showing enlarged inguinal lymph nodes reaching 15 mm in the shortest axis.
The patient received packed red blood cells transfusions to maintain his hemoglobin level around 8 mg/dL. He was also given intravenous fluids to treat the hypercalcemia and received one dose of pamidronate, which helped to lower the calcium level to as low as 11 mg/dL over the next few days.
The presence of massive splenomegaly and diffuse lymph node enlargement was concerning lymphoma. Further workup showed anemia of chronic disease and elevated vitamin D 1,25(OH)2 levels (with low PTH levels). Anti-nuclear antibody, HIV test, monospot test, and purified protein derivative (PPD) were negative. Additional studies are listed in Table
Laboratory findings.
Parameter | Value |
---|---|
Total protein (g/dL) | 8.1 (6–8.3) |
Albumin (g/dL) | 3.2 (3.0–5.5) |
Serum iron ( |
34 (35–150) |
Total iron binding capacity ( |
241 (260–400) |
Ferritin (ng/mL) | 608 (30–400) |
Percent saturation (%) | 14.1 (15–50) |
Reticulocyte count (%) | 1.88 (0.5–1.5) |
ESR (mm/h) | 93 (0–10) |
Vitamin B12 (pg/mL) | 215 (243–894) |
Lactate dehydrogenase (IU/L) | 97 (60–200) |
Inorganic phosphorus (mg/dL) | 2.5 (2.1–4.9) |
Intact PTH (pg/mL) | 5 (15–65) |
PTH related protein (pg/mL) | 22 (14–27) |
Thyroid stimulating hormone ( |
2.72 (0.27–4.2) |
Vitamin D 1,25(OH)2 total (pg/mL) | 248 (18–72) |
Vitamin D 25-OH total (ng/mL) | 26 (30–100) |
Uric acid (mg/dL) | 6.3 (4.8–8.7) |
Serum protein electrophoresis (SPEP) | Normal |
Free kappa/lambda ratio | 1.01 (0.26–1.65) |
Urine protein electrophoresis (UPEP) | Normal |
Nonnecrotizing granulomata. Low- (a) and high-magnification (b) photomicrograph of a section from an inguinal lymph node. It shows that the lymph node is replaced by numerous small compact nonnecrotizing granulomata.
Four weeks after the initiation of therapy, the patient’s calcium level was 10.3 mg/dL, along with a hemoglobin of 9.4 g/dL and a creatinine of 1.38 mg/dL. 2 weeks later, ACE level was 31 mg/dL. 8 weeks after hospital discharge, a fluorine-18 fluorodeoxyglucose (FDG) PET imaging, done while the patient was still on treatment, showed no focal FDG avid lesions, along with stable non-FDG avid thoracic and abdominal adenopathy. The patient continued to be in good health 9 months after his diagnosis and had shown no progression of sarcoidosis.
Sarcoidosis is a chronic idiopathic granulomatous disease which can affect all age groups [
Sarcoidosis can be asymptomatic in some patients. If present, symptoms are both systemic (fever, weight loss, and fatigue) and/or organ-specific (shortness of breath, chest pain, and cough) [
The diagnosis of sarcoidosis is based on criteria from the American Thoracic Society (ATS), the European Respiratory Society (ERS), and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) [
Some patients with sarcoidosis are not disabled by the illness and therefore do not require treatment [
Splenic involvement in sarcoidosis is defined as the histologic presence of noncaseating granulomas in the spleen. Autopsy studies show that the spleen is the second most commonly affected organ in sarcoidosis, with the lung being first [ Myelofibrosis (primary or secondary). Chronic myeloid leukemia. Lymphoma (usually indolent). Hairy cell leukemia. Gaucher disease. Amyloidosis. Beta thalassemia major. Schistosomiasis. Kala-azar (visceral leishmaniasis). Sarcoidosis (rarely). Hyperreactive malarial splenomegaly syndrome (tropical splenomegaly syndrome). AIDS with mycobacterium avium complex. Splenic vein thrombosis.
Splenic involvement in sarcoidosis is usually asymptomatic, although left upper quadrant pain is occasionally present. Patients with splenomegaly may have a higher incidence of constitutional symptoms and more disseminated disease [
The crux of sarcoidosis is its ability to masquerade as other diseases, most significantly lymphoma. As such, for clinicians, distinguishing these entities can make the difference between life and death for patients. In addition to the nonspecific clinical, radiological, and histological features in sarcoidosis, the lymphocyte activation and the reticuloendothelial system involvement (lymph nodes, spleen, and liver) make the distinction of sarcoidosis from lymphoma extremely challenging. In fact, hypercalcemia and increased serum ACE levels have also been described in patients with lymphoma [
In some cases, the coexistence of “true” sarcoidosis and lymphoproliferative disease has been reported in the literature. In most of these cases, sarcoidosis preceded the diagnosis of lymphoma, but in few other reports, lymphoproliferative disease occurred first [
FDG PET imaging remains an essential modality in the management of lymphoma. One of many advantages it offers over conventional imaging is the ability to detect occult lesions. However, its specificity is limited by multiple false-positive conditions, including infections, inflammations, and sarcoidosis [
This report illustrates an unusual case of sarcoidosis that presented as bicytopenia, hypercalcemia, diffuse lymphadenopathy, and massive splenomegaly, mimicking lymphoma. Physicians should be aware of this atypical presentation and accordingly should consider sarcoidosis in their differential diagnosis, after excluding other worrisome diagnoses, such as lymphoma.
Informed consent was obtained from the patient for publication of this case report and any accompanying images.
The authors declared no conflict of interests.