POEMS syndrome

Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.

POEMS syndrome (also known as Crow-Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare medical syndrome named for its main clinically recognizable features: Polyneuropathy (peripheral nerve damage), Organomegaly (abnormal enlargement of organs), Endocrinopathy (damage to hormone-producing glands)/Edema, M-protein (an abnormal antibody) and Skin abnormalities (including hyperpigmentation and hypertrichosis).

All patients have polyneuropathy, which usually manifests itself with abnormal and reduced sensation and decreased power in the legs and arms. The condition may be painful and requiring analgesia.

A 2003 study[1] followed a series of 99 patients and attempted to delineate criteria for the diagnosis of POEMS syndrome. According to their study, both two major criteria and at least one minor criterion are required for the diagnosis:

In addition, their study showed a number of associations, the relevance of which to diagnosis was unclear: clubbing, weight loss, thrombocytosis, polycythemia and hyperhidrosis. Possible associations included pulmonary hypertension, restrictive lung disease, a thrombotic diathesis, arthralgia, cardiomyopathy (systolic dysfunction), fever, low vitamin B12 levels and diarrhea.[1]

While the main features of the disease have been described, the exact mechanism of the disease has remained elusive. The paraprotein alone is insufficient to explain the multi-organ features, and various cytokines produced by plasmacytoma cells have therefore been linked with the features of POEMS syndrome, specifically interleukin 1ß, interleukin 6 and TNFa. There also seems to be a role of vascular endothelial growth factor (VEGF), given the angiogenetic changes in many tissues.[1]

Dispenzieri et al[1] list numerous treatments, the effect of many of which is almost anecdotal. Prednisolone and alkylating agents are the most commonly used. The Mayo Clinic group attempted hematopoietic stem cell transplantation in sixteen patients; one patient died during the treatment, and in several others respiratory problems were unmasked in the course of the procedure. The outcome of the treatment, though, was good.

[tubepress mode='tag', tagValue='POEMS syndrome']