plasma cell leukemia
Introduction
Introduction to plasma cell leukemia Plasma cell leukemia (PCL) is a rare type of acute leukemia. Plasma cell leukemia is divided into two categories: primary plasma cell leukemia and secondary plasma cell leukemia. basic knowledge The proportion of illness: this disease is rare, the incidence rate is about 0.0004% - 0.0009% Susceptible people: no specific population Mode of infection: non-infectious Complications: multiple myeloma
Cause
Plasma cell leukemia
(1) Causes of the disease
Primary PCL causes multiple myeloma, secondary PCL can be transformed from multiple myeloma, lymphoma, chronic lymphocytic leukemia, macroglobulinemia.
(two) pathogenesis
Pathological features Abnormal infiltration of plasma cells can spread throughout the body tissues, such as plasma cells infiltrating the bone marrow to destroy the peripheral blood barrier of the bone marrow, entering the material circulation to produce plasma cell leukemia.
Prevention
Plasma cell leukemia prevention
1. Do not abuse drugs. Care should be taken when using chloramphenicol, cytotoxic anticancer drugs, immunosuppressive drugs, etc., and must be guided by a doctor. Do not use or abuse them for a long time. In addition, use as little or no hair dye as possible. American researchers have found that women who use hair dyes (especially in large quantities) are 3.8 times more likely to develop leukemia than the average person. Barbers, beauticians, and cosmetic surgeons who are often exposed to hair dyes are also potentially harmful.
2, pay attention to food hygiene. Because vegetables, fruits and other foods containing chemical fertilizers and pesticides are digested and absorbed into the blood after consumption, it is easy to destroy the normal hematopoietic function of the bone marrow and cause disease. Therefore, vegetables and fruits should be cleaned before consumption, and the residual amount of chemical fertilizers and pesticides should be minimized.
3, for high-risk groups of leukemia should do a regular census work, pay special attention to leukemia police signs and early symptoms.
4, to avoid exposure to certain carcinogens, do a good job of occupational protection and monitoring: such as in the production of phenol, chlorobenzene, nitrobenzene, spices, pharmaceuticals, pesticides, synthetic fibers, synthetic rubber, plastics, dyes, etc., pay attention to Avoid contact with harmful and toxic substances.
5, to avoid exposure to excessive X-rays and other harmful radiation, personal protection for those engaged in radiation work, pregnant women and infants should pay special attention to avoid exposure to radiation.
6, prevention and treatment of various infections, especially viral infections: such as C-type RNA virus.
Complication
Plasma cell leukemia complications Complications Multiple myeloma
Same as multiple myeloma.
Symptom
Symptoms of plasma cell leukemia Common symptoms Awareness disorder Lymph node enlargement
Plasma cell leukemia has clinical manifestations of acute leukemia such as anemia, hemorrhage, secondary infection, extramedullary infiltration, etc. Relatively speaking, the age of primary plasma cell leukemia is relatively mild, and the liver, spleen and lymph nodes are more prominent. No M component or M component is limited, osteolytic lesions are absent or less, and chemotherapy response is relatively good. Secondary plasma cell leukemia is the end stage of multiple myeloma, often anemia, heavier bleeding, M component The level was significantly elevated, the osteolytic lesion was severe, and it was resistant to chemotherapy. Among the 24 cases reported by Vela-Ojeda, 62% were women, 88% were anemia, 54% were bleeding, 42% were dysfunction, 71% were weight loss, and the liver was 25 %, the spleen is 21% larger.
Examine
Plasma cell leukemia
1. Peripheral plasma cells 2 × 109 / L, or classification 20%, which may have primitive, immature plasma cells or myeloma cells, white blood cell count can be increased, can also be reduced or normal, often accompanied by anemia and (or) Thrombocytopenia, SPCL patients with more complete reduction of blood cells, increased white blood cells accounted for 62%, thrombocytopenia accounted for 71%.
2. Bone marrow like bone marrow hyperplasia is mostly active, plasma cells, including primitive, immature plasma cells or myeloma cells are often >30%, plasma cell morphology is often abnormal, glycogen and acid phosphatase staining positive.
3. Immunophenotype The two types of PCL have similar immunophenotypes, all of which are positive for CD38, PCA-1, CD56, and CD85, while CD19, CD20, CD22, CD24, and HLA-DR are negative or weakly positive.
4. Blood biochemistry and immunological examination SPCL often have abnormal renal function (creatinine, urea nitrogen is significantly increased), blood creatinine increased about 50%, serum albumin decreased, globulin increased, blood calcium also increased 37%, immunoglobulin quantification, serum protein electrophoresis and serum immunoelectrophoresis showed elevated immunoglobulin and/or mono-light chain elevation. There was no single immunoglobulin in the serum of PPCL, and most of the kidney function. Normal, elevated lactate dehydrogenase accounted for 58%, and 2-microglobulin increased by 71%.
5. Urinary-peripheral protein positive accounted for about 79%.
6. Imaging examination Most of SPCL patients have general osteoporosis, osteolytic lesions and pathological fractures. Skull, ribs, spine vertebral bodies and pelvis are more common. PPCL usually has no bone abnormalities.
7. B-ultrasound showed liver and spleen, swollen lymph nodes.
Diagnosis
Diagnosis and differentiation of plasma cell leukemia
Diagnostic criteria
1. Present the clinical manifestations of leukemia or the clinical manifestations of myeloma.
2. Peripheral blood leukocyte classification, plasma cells 20% or absolute number 2 × 109 / L.
3. Bone marrow plasma cell proliferation, the original and immature plasma cells increased significantly, accompanied by abnormal morphology.
The above criteria are similar to the diagnostic criteria proposed by Kyle abroad. The morphological features of SPCL plasma cells (including myeloma cells) are obvious, and can usually be diagnosed without immunophenotyping. The diagnosis of PPCL should be done in an immunophenotype if conditions permit. Check to determine that the original and naive cells are derived from plasma cells.
Diagnostic evaluation: PCL is mainly based on the number of peripheral plasma cells, SPCL, due to the existence of MM disease, there is no difficulty in diagnosis, and PPCL, must be excluded before diagnosis of reactive plasmacytosis.
Differential diagnosis
1. Reactive bacterial cell hyperplasia (RP) identification point
1RP has primary disease, including various serious infections (most common tuberculosis), tumors, rheumatic diseases, chronic liver diseases.
There are few plasma cells in the peripheral blood of 2RP, mainly in the bone marrow, and the number is mostly less than 10% of nucleated cells, the vast majority <20%, and the morphology is normal plasma cells.
3RP disappeared with the relief of primary disease.
2. Acute myeloid leukemia (AML) The clinical manifestations of PPCL are very similar to AML, mainly based on morphology, cytochemical staining and immunophenotypic differentiation, due to the morphology of plasma cells, including primitive and immature plasma cells, and primitive granulocytes. Usually not easy to be confused; cytochemical staining, such as peroxidase, AML positive, and PPCL negative, atypical can be distinguished by immunophenotypic detection, plasma cells are CD38, PCA-1 positive, while AML original cells are CD33, CD13 is positive.
3. Acute lymphoblastic leukemia (ALL) PPCL occasionally needs to be differentiated from ALL. When the original and naive cells are in atypical state, they can be differentiated by immunophenotype. B-ALL is CD19, CD20, CD10 positive, T- ALL was positive for CD2, CD5, and CD7, while the original plasma cells were positive for CD38 and PCA-1.
