What Is POEMS Syndrome?

POEMS syndrome is a multi-system disease associated with plasma cell disease. Clinically, polyneuropathy, organomegaly, endocrinopathy, Moc Skin changes are characterized, and the combination of the English prefix of various lesion terms is named POEMS syndrome.

Basic Information

nickname: Grow-Fukase syndrome
English name: POEMS Syndrome

Visiting department: Hematology
Common symptoms: Acupuncture or sock-like sensations in the extremities with muscle weakness

etiology and pathogenesis of poems syndrome

The etiology and pathogenesis of POEMS syndrome is unknown, and it is currently thought to be related to vascular endothelial growth factor (VEGF), pro-flammatory cytokines, matrix metalloproteinase (MMP) and HHV-8 infection (1, 2).

clinical manifestations of poems syndrome

The disease is rare, with a median age of onset of 51 years and more men than women, 2 to 3: 1. The onset is insidious, and as the disease progresses, the clinical manifestations gradually increase, and multiple systems can be involved.

Multiple peripheral neuropathy

Seen in all patients, most of them are the first symptoms, which are characterized by chronic, symmetrical, progressive sensation and motor dysfunction. From the end of the foot, they gradually show abnormalities like acupuncture or gloves and socks in the limbs, and muscle weakness.

2. Autonomic dysfunction

Autonomic nerve dysfunction may occur in some patients, including hyperhidrosis, hypotension, impotence, diarrhea, or constipation.

3. Have organ enlargement

Mainly manifested as liver, splenomegaly and lymphadenopathy. Liver enlargement accounts for 24% to 78%, splenomegaly accounts for 22% to 52%, and lymphadenopathy accounts for 11% to 24%.

4. Endocrine changes

Endocrine system abnormalities are characteristic of POEMS syndrome. Diabetes, hypothyroidism, impotence in men, and amenorrhea in women are more common.

5. Skin changes

50% to 90% of patients have skin changes. Among them, focal or systemic skin pigmentation is the most common. Other manifestations are edema, hairy (usually limited to limbs, chest and face), sweating, clubbing fingers, Raynaud Phenomenon, hemangioma and white nails.

poems syndrome laboratory test

Lymph node biopsy

The pathological results can be Castleman's disease (megalymph node hyperplasia), reactive hyperplasia, and chronic lymphadenitis.

2. Peripheral nerve biopsy

The pathology was demyelinating changes.

3.M protein and bone marrow abnormalities

All patients had M protein, mostly IgG or IgA, lambda light chain type. Bone marrow plasma cells can be slightly increased, and flow cytometry can be identified as clonogenic.

4. Other

X-rays of most patients show characteristic osteosclerosis with or without osteolytic damage. About 10% to 20% of patients coexist with Castleman disease. In addition, the disease can still have the following manifestations: low fever, wasting, edema, serous fluid, optic nerve papillary edema, pulmonary hypertension, clubbing, Raynaud's phenomenon, thrombocytosis, and elevated serum VEGF (vascular endothelial growth factor) .

poems syndrome diagnosis

As this disease is rare, there is no unified diagnostic standard at present. The following domestic standards can be used as a reference:

Multiple peripheral neuropathy

2. Organ swelling (most common in liver and spleen)

3. Have endocrine disease

4. Have M protein or plasmacytoma

5. Has skin lesions

6.Bone sclerosis

Among the above items, M protein and peripheral neuropathy are the main diagnostic criteria, and the rest are secondary criteria. Studies have suggested that bone sclerosis, Castleman disease, and optic disc edema are also secondary diagnostic criteria. A typical case has the above five lesions. The diagnosis of POEMS syndrome must have two primary diagnostic criteria and at least one secondary criterion.

poems syndrome treatment

There is no standard treatment for this disease.

1. Support symptomatic treatment

Patients should pay attention to rest. Diabetic patients should pay attention to diet, if there is edema, low-salt diet. For patients with respiratory muscle weakness or pulmonary hypertension, continuous oxygen or continuous positive pressure ventilation is necessary. If hormone deficiency, hormone replacement therapy is performed, and patients with peripheral neuropathy can be given physical therapy and nutritional neurotherapy.

2. Radiation therapy

If a single or multiple osteosclerotic lesions are found in a confined area, radiation therapy should be performed. If it is a systemic osteosclerotic lesion, comprehensive treatment is required.

3. Immunosuppressive

Cyclophosphamide: Cyclophosphamide alone or in combination with prednisone can provide clinical response in 40% of patients. For patients with more serious conditions who cannot receive stem cell transplantation immediately, or patients whose condition changes rapidly while waiting for peripheral blood stem cell transplantation, cyclophosphamide (combined with prednisone or alone) can be given intravenously.

L-phenylalanine mustard: It is one of the most effective drugs in the treatment of plasma cell diseases, and corticosteroids are effective in 40% of patients with POEMS syndrome, but it should be avoided before collecting stem cells.

Vincristine, azathioprine, cyclosporine and methotrexate can also be used.

4. Glucocorticoids

Prednisone can be used in general patients. Severe patients can be treated with methylprednisolone shock therapy. After improvement, oral prednisone can be used instead. There are no prospective studies supporting the efficacy of corticosteroids in POEMS syndrome. However, there are case reports and personal experience that glucocorticoids are effective. At least 15% of patients have improved clinical symptoms after using corticosteroids alone, and another 7% of patients have disease control. Therefore, treatment with corticosteroids can be considered a palliative therapy.

5. Tamoxifen

It has been reported that patients who have failed to use prednisone or cyclophosphamide switched to tamoxifen. The clinical symptoms have changed dramatically, muscle strength has improved significantly, and life can take care of themselves. The specific mechanism of action is unknown, and it is currently thought that it may be related to its antitumor effect .

6. Chemotherapy

MP, CP, COP, or MOP programs can alleviate clinical symptoms to varying degrees.

7. New Drug

It has been reported that the plasma VEGF level (vascular endothelial growth factor) in patients with POEMS syndrome is significantly higher than that in normal people, which is likely related to plasma cell tumor secretion. It is expected that antibodies to VEGF can play a certain role. Badros et al reported a case of patients with POEMS syndrome treated with bevacizumab. As a result, the patient's neuropathy and edema improved significantly, but due to the lack of application experience, further observation is needed.

8. High-dose chemotherapy combined with hematopoietic stem cell transplantation (ASCT)

9. Plasma exchange

Combined with immunosuppressive therapy, clinical symptoms can be alleviated to varying degrees.

10. gamma globulin

poems syndrome prognosis

POEMS syndrome is a chronic progressive disease, and its prognosis depends on the nature and condition of the associated disease. Current research suggests that the median survival of patients from the first symptom is 5 to 7 years. Solitary osteolytic bone The prognosis of the injured is better, the clinical symptoms of POEMS syndrome have no effect on survival, and the prognosis of those with plasma cell lesions in the bone marrow is poor. The continuous deterioration of neuropathy is a common outcome and cause of death in POEMS syndrome, and bone marrow failure secondary to disease progression and chemotherapy is a common cause of death in multiple myeloma. Patients die mainly from disease progression, pneumonia, sepsis, Stroke and multiple myeloma. However, in recent years, with the in-depth study of the biology and application of cytokines and anti-antibodies, and the simultaneous assistance of traditional Chinese medicine and integrated traditional Chinese and western medicine treatment, it is expected to greatly improve the prognosis of POEMS syndrome. ^[2-11]