A 77-year-old male with systolic heart failure (EF 28%), hypothyroidism, and CKD stage 3 presented with dyspnea on exertion and paresthesias in all four extremities. Physical exam revealed anasarca. CT scan of chest/abdomen/pelvis revealed large volume ascites, pericardial and pleural effusions. Transthoracic echocardiogram revealed large pericardial effusion without evidence of tamponade physiology. Initial labs revealed hemoglobin 12.4g/dL, BUN 54 mg/dL, Cr 4.01 mg/dL, Albumin 3.6g/dL. Paracentesis was performed and 10 liters of ascites was removed; SAAG of 1.8 indicated portal hypertension to be the etiology of the ascites. Abdominal ultrasound revealed hepatic steatosis, normal kidneys, and patent portal vein. Viral hepatitis panel and HIV screen were negative. Serum immunofixation revealed monoclonal gammopathy with IgM lambda spike. Serum free kappa/lambda ratio was normal. Abdominal fat pad biopsy ruled out amyloidosis. Bone marrow biopsy demonstrated no plasma cell dyscrasia. Skeletal survey did not show bone lytic lesions. Serum vascular endothelial growth factor (VEGF) level was found to be elevated on ELISA (120). The patient fulfilled criteria for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) based on polyneuropathy, monoclonal IgM lambda gammopathy, elevated serum VEGF and volume overload. The patient’s hospital course was complicated by perforated cholecystitis, peritonitis, and liver abscess, which was treated with drainage and antibiotics.
Discussion: POEMS syndrome is a rare multisystem disorder with unclear etiology and variable clinical presentation. Diagnosis of POEMS syndrome is challenging. The Mayo Clinic criteria for diagnosis of POEMS syndrome require peripheral neuropathy and monoclonal plasma cell disorder. In addition, patients must fulfill 1 of 3 major criteria (osteosclerotic bone lesions, elevated VEGF or Castleman’s disease), and 1 of 6 minor criteria (endocrinopathy, skin changes, organomegaly, extravascular volume overload, thrombocytosis/polycythemia or papilledema). Although no standard treatment currently exists for POEMS syndrome, early therapy may begin upon diagnosis, ideally prior to multi-organ involvement. Treatment may include hematopoietic stem cell transplant, radiation, and chemotherapy (i.e. Melphalan plus dexamethasone or agents such as Lenalidomide, Thalidomide or Bortezomib). Unfortunately, our patient’s disease was advanced on presentation; he agreed only to supportive therapy.
Conclusions: POEMS syndrome is often misdiagnosed or under-diagnosed due to its rarity and complexity. This diagnosis should be considered in patients with multi-organ system dysfunction of unclear etiology.
To cite this abstract:Bernabe C, Sun S, Harrington M. Poetry in Diagnosis: A Case of Poems Syndrome. Abstract published at Hospital Medicine 2015, March 29-April 1, National Harbor, Md. Abstract 455. Journal of Hospital Medicine. 2015; 10 (suppl 2). https://www.shmabstracts.com/abstract/poetry-in-diagnosis-a-case-of-poems-syndrome/. Accessed May 26, 2019.