China Multiple Myeloma Working Group
Multiple myeloma is one of the most common malignancies of the hematologic system. In recent years, rapid progress has been made in the diagnosis, treatment, and efficacy criteria of multiple myeloma due to the emergence of new drugs that have improved efficacy. In view of this, the Hematologist Branch of the Chinese Medical Association has convened some experts from across the country to develop this guideline.
I. Definition Ma Lan, Department of Hematology, Wuxi Hospital of Traditional Chinese Medicine
Multiple myeloma is a malignant proliferative disease of plasma cells in which clonal plasma cells in the bone marrow proliferate abnormally and secrete monoclonal immunoglobulins or their fragments (M proteins) and cause related organ or tissue damage (ROTI). Common clinical manifestations are bone pain, anemia, renal insufficiency, and infection.
II. Clinical manifestations
The most common symptoms of multiple myeloma are those associated with anemia, renal insufficiency, infection or bone destruction. Commonly, there are.
1. Skeletal symptoms: bone pain, local masses, pathological fractures, and may be combined with paraplegia.
2. immune deficiency: recurrent bacterial pneumonia and/or urinary tract infections, sepsis; viral infections with herpes zoster are common.
3. Anemia: orthocytic orthochromic anemia; rarely combined with leukopenia and/or thrombocytopenia.
4. Hypercalcemia: vomiting, weakness, confusion, polyuria or constipation.
5. Renal impairment: Light chain tubular nephropathy is the most common cause of renal failure.
6. Hyperviscosity syndrome: there may be dizziness, vertigo, blurred vision, tinnitus, and sudden onset of impaired consciousness, finger numbness, insufficient coronary artery blood supply, and chronic heart failure. In addition, the M component of some patients is cold globulin, which causes microcirculatory disorders and Raynaud’s phenomenon.
7. Others: Those with amyloidosis lesions may show hypertrophy of the tongue, enlargement of the parotid gland, enlarged heart, diarrhea or constipation, enlarged liver and spleen and peripheral neuropathy; advanced patients may also have bleeding tendency.
Table 1 Myeloma-related organ or tissue damage (ROTI)
Elevated blood calcium levels
Calibrated serum calcium above the upper limit of normal of 0.25 mmol/L [1 mg/dL] or >2.8 mmol/L [11.5 mg/dL]
Impaired renal function
Blood creatinine >176.8 μmol/L [2mg/dL]
Anemia
Hemoglobin <100g/L or more than 20g/L below normal
Bone destruction
Osteolytic damage or osteoporosis with compression fracture
Other
Symptomatic hyperviscosity, amyloidosis, recurrent bacterial infections (≥2 times/year)
III. Diagnostic criteria, typology, staging and differential diagnosis
(I) Diagnosis
1.Diagnostic criteria
Main criteria.
①Tissue biopsy proves the presence of plasmacytoma or bone marrow smear examination: plasma cells > 30%, often accompanied by morphological changes.
② Monoclonal immunoglobulin (M protein): IgG>35g/L, IgA>20g/L, IgM>15g/L, IgD>2g/L, IgE>2g/L, monoclonal K or λ light chain in urine>1g/24 hours, and exclude amyloidosis.
Secondary criteria.
①Bone marrow examination: 10% to 30% plasma cells.
② Presence of monoclonal immunoglobulin or its fragments, but below the above criteria.
③Osteolytic damage and/or extensive osteoporosis on x-ray.
④Decreased amount of normal immunoglobulins: IgM