About takatsuki syndrome
What is takatsuki syndrome?
POEMS syndrome is an extremely rare multisystem disorder. POEMS is an acronym that stands for (P)olyneuropathy, disease affecting many nerves; (O)rganomegaly, abnormal enlargement of an organ; (E)ndocrinopathy, disease affecting certain hormone-producing glands that help to regulate sexual function, and certain metabolic functions; (M)onoclonal gammopathy or M proteins; and (S)kin abnormalities. Common symptoms include progressive weakness of the nerves in the legs and arms, an abnormally enlarged liver and/or spleen (hepatosplenomegaly), enlarged lymph nodes, abnormally darkening of the skin (hyperpigmentation), thickening of the skin and excessive hair growth (hypertrichosis). Endocrine abnormalities such as failure of the ovaries and testes (gonads) to function properly (primary gonadal failure) and diabetes mellitus may be present. Specific endocrine abnormalities associated with POEMS syndrome vary from case to case. Other important features of the disease include swelling around the optic nerve (papilledema), abnormal fluid retention, which may occur in the ankles (edema), the abdominal cavity (ascites), or around the lungs (pleural effusions), painless scars on bone x-ray (osteosclerosis), and an elevated platelet count (a blood cell responsible for clotting). Some patients may also have abnormalities in the lung function tests, which may not be noticed because of their inability to walk well due to their neuropathy.
POEMS syndrome is associated with a group of disorders known as monoclonal gammopathies or plasma cell dyscrasias. These disorders are characterized the uncontrolled growth of a single clone (monoclonal) of plasma cells, which results in the abnormal accumulation of M-proteins (also known as immunoglobulins) in the blood. Immunoglobulins in health fight infection. However, the specific role M-proteins play and the exact cause of POEMS syndrome is unknown. Research would suggest that a chemical called VEGF (vascular endothelial growth factor) plays an important role in this disease.
What are the symptoms for takatsuki syndrome?
- Polyneuropathy. Numbness, tingling and Weakness in your legs — and over time, maybe in your hands — and difficulty breathing. This is an essential feature in the diagnosis of POEMS syndrome.
- Organomegaly. Enlarged spleen, liver or lymph nodes.
- Endocrinopathy/edema. Abnormal hormone levels that can result in an underactive thyroid (hypothyroidism), diabetes, sexual problems, fatigue, swelling in your limbs, and problems with metabolism and other essential functions.
- Monoclonal-protein. Abnormal bone marrow cells (plasma cells) that produce a protein (monoclonal protein) that can be found in the bloodstream. This is an essential feature in the diagnosis of POEMS syndrome. Monoclonal-protein is often associated with unusual bone hardening or thickening.
- Skin changes. More color than normal on your skin, red spots, possibly thicker skin, and increased facial or leg hair.
What are the causes for takatsuki syndrome?
The cause of POEMS syndrome isn't known. But people with POEMS syndrome have an increased number of plasma cells. These cells produce an abnormal amount of a type of protein, which can damage other parts of the body.
What are the treatments for takatsuki syndrome?
Treatment for POEMS syndrome might improve your symptoms but doesn't cure the condition. Treatment options include radiation therapy, chemotherapy and stem cell transplant. The outlook for people with POEMS syndrome has improved greatly over the past decade or so.
What are the risk factors for takatsuki syndrome?
No kown risk factors
Is there a cure/medications for takatsuki syndrome?
Takatsuki syndrome, also known as POEMS syndrome, is a blood disorder that occurs in the body. In this uncommon circumstance, abnormal plasma cells cause an increase in antibody protein levels in the blood. Too many of these proteins in the blood can cause nerve damage and the malfunction of several organ systems.
POEMS syndrome is uncommon and can be fatal. Osteosclerotic myeloma, Crow-Fukase syndrome, and Takatsuki syndrome are other names for it.
POEMS syndrome treatment focuses on symptom management. Therapy may consist of:
1. Chemotherapy: Anti-cancer medications will either slow or destroy the aberrant plasma cells. Treatment is comparable to that of multiple myeloma, a kind of plasma cell malignancy. These treatments are exceedingly successful and, in most cases, well tolerated, with few adverse effects. Immunotherapy medications assist your immune system in killing off the harmful plasma cells
2. Radiation therapy: High amounts of radiation are used by doctors to kill harmful plasma cells.
3. Physical therapy: This treatment may aid in mobility concerns caused by neuropathy.
4. Other medicines Steroids and/or diuretics (to reduce swelling) may be prescribed as well.
Numbness, tingling and weakness in your legs,Enlarged spleen, liver or lymph nodes,Abnormal hormone levels,Abnormal bone marrow cells,Skin changes
Steroids and/or diuretics