Plasma cell leukemia was first reported in 1904 and accounts for 1%-2% of multiple myeloma (MM). Clinical manifestations of plasma cells in peripheral blood >20% or absolute value of 2.0×109/L; bone marrow image plasma cells significantly proliferated, primitive and naive plasma cells significantly increased, with abnormal morphology.
Plasma cell leukemia is divided into two types: 1. Primary plasma cell leukemia (PPCL): occurs in patients without a history of MM. At the onset of the disease, peripheral blood plasma cells are >20%, or the absolute value of peripheral blood plasma cells is ≥2.0×109/L with morphological abnormalities. The clinical presentation is similar to that of acute leukemia.
2, secondary plasma cell leukemia (SPCL): most people secondary to MM, a few secondary to macroglobulinemia, lymphoma, chronic lymphocytic leukemia and amyloidosis. The clinicopathology of SPCL secondary to MM is basically similar to that of MM and is an end-stage manifestation of MM.
The patient, a male, was 74 years old. He was seen in a hospital in Beijing in October 2005 because of weakness, dizziness, and low back pain, which worsened progressively. The diagnosis of plasma cell leukemia was confirmed by routine blood tests, peripheral blood leukocyte classification (54% plasma cells), and bone marrow morphology. o No immunoglobulin electrophoresis was performed at the time of diagnosis, and based on the clinical symptoms at the time of presentation, plasma cell leukemia secondary to multiple myeloma was considered. He had a history of diabetes mellitus and hypertension, and used insulin to control blood glucose and oral antihypertensive drug valsartan to control blood pressure, both of which were satisfactory. After the initial diagnosis, the patient was in complete remission after two MP regimens of chemotherapy, and the anemia was corrected and the symptoms were relieved. 1.5 years later, the above-mentioned symptoms reappeared, and the examination suggested relapse.
The patient was admitted to our hospital on June 29, 2007 for further treatment. Bone marrow morphology on admission showed: bone marrow proliferation was obviously active, plasma cells accounted for 36%, of which 26% were primitive and naive plasma cells, blood routine: leukocytes 3.74×10^9/L, hemoglobin 54g/L, platelets 79×10^9/L, peripheral blood leukocyte classification did not see naive plasma cells; renal function: inosine 154umol/L, urea nitrogen 12mmol/L. Blood immune Blood immunoglobulin quantification showed: IgG19.6g/L, IgA0.089g/L, IgM0.055g/L, κ light chain 2730mg/dl, λ light chain 30mg/dl, serum protein electrophoresis showed monoclonal bands, urinary immunoglobulin disease, hypertension (grade 3, very high risk). He was given chemotherapy with VAD regimen on July 2, 2007, and the bone marrow was rechecked 18 days after chemotherapy: active proliferation, 17% plasma cells.
1. Bone marrow: hypoproliferation, plasma cells accounted for 23%.
2.Blood count: leukocytes 2.37×10^9/L, hemoglobin 63g/L, platelets 108×10^9/L, 3.peripheral blood leukocytes classification did not see naïve plasma cells, 4.renal function: inosine 125umol/L, urea nitrogen 14.43mmol/L. After 4 cycles of bortezomib combined with MP, M2 and other regimens of chemotherapy to evaluate the condition: blood immunoglobulin Blood immunoglobulin quantification was in the normal range, urine Benzedrine was negative, and no plasma cells were seen in the bone marrow. The patient was then given four cycles of bortezomib combined with MP for consolidation chemotherapy, and her condition was evaluated to be in complete remission.
5.Anemia symptoms improved significantly, and hemoglobin could be maintained at 80-90 g/L without blood transfusion. 6.Renal function returned to normal.
7.The minimum platelet drop to 20×10^9/L, without platelet transfusion, and can return to normal after stopping the drug.
8.After 4 cycles of chemotherapy, mild numbness of fingers and toe tips appeared, which was relieved after 1 cycle of methylcobalamin treatment, without other significant adverse effects.