Blood Disorders » POEMS Syndrome

POEMS Syndrome

POEMS Syndrome - invariably referred to as Crow-Fukase syndrome, PEP syndrome, or Takatsuki disease - is a quite rare multi-system disorder (a sort of blood disorder), which seem to affect humans without a known cause. In fact, its name itself is an acronym of its symptoms - (P)olyneuropathy (disease affecting the peripheral nervous system, (O)rganomegaly (beyond normal enlargement of organs), (E)ndocrinopathy (disease affecting hormone-secreting glands)/(E)dema, (M)-protein (an abnormal antibody) and (S)kin abnormalities (such as hypertrichosis and hyperpigmentation).  
    
Common symptoms of the disease, as comprehensible to a common man, include progressive weakness of legs and arms, abnormally enlarged spleen or liver, excessive hair growth, and hyperpigmentation (darkening of skin). Dysfunction of gonads and Diabetes mellitus I may also occur, but it varies from case to case.  

POEMS syndrome is generally linked to a collection of disorders called plasma cell dyscrasias or monoclonal gammopathies. These medical conditions are characterized by the unbridled multiplication of monoclonal (single clone) plasma cells, which is the main reason behind the abnormal presence of immunoglobulin M (M-Proteins) in the blood. M-proteins are supposed to fight the presence of foreign bodies in the blood, but researchers feel that they, if in an excess amount, can trigger a medical condition such as the POEMS syndrome. But the exact role and causes of the disease still remains elusive to expert’s knowledge.

Diagnosis

As POEMS Syndrome is a rare occurrence, it is not uncommon that some doctors at least may fail to read its symptoms in the first diagnosis. In other words, the disease requires expertise and knowledge from the physician’s part to be spotted at an earlier stage itself. Occurrence of other diseases may also complicate the diagnosis efforts. The popular diagnostic method, as followed across the globe, is to check for an excess presence of plasma cells in the blood and urine. Once diagnosed, the treatment is usually decided on the basis of the severity or stage of the disease at any given point of time.

Treatment

The common treatments include radiation therapy, chemotherapy, corticosteroids, hormone replacement therapy bone marrow transplant, and physical therapy. Radiation therapy is employed if the multiplication of plasma cell clone is minimal, while chemotherapy is used to contain advanced stages. Hormone replacement therapy is prescribed for acute hormonal abnormalities and bone marrow transplant as a more effective cure for some of the symptoms. But the latter is costlier and has the risk of side effects. In some acute cases, the patients may suffer from nerve damages (neuropathy). In such cases the only treatment possible is to prevent further damage is to kill the plasma cell clone (osteosclerotic myeloma). But the recovery of the patient depends very much on the severity of the disease at the time of initiation of the treatment.

Final Word

The cure to POEMS syndrome depends heavily on how early the disease is being diagnosed. Even after the symptoms subside after treatment, the patients need to undertake a lifelong follow-up as the chances for relapses are high and hence cannot be ruled out. Also, it is important to get the treatment and diagnosis done from the best doctors in the league. Remember, as it is a rare disease, not everyone may not be having the expertise to spot the beast. Good Luck!

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