Clinical analysis of 15 patients with Waldenstr?m macroglobulinemia received bortezomib-based treatment / 药学实践杂志

Objective To summarize the clinical experience of bortezomib-based treatment for Waldenstr?m macroglobu-linemia and evaluate the therapeutic efficacy and safety .Methods The clinical data were collected for 15 patients with Waldenstr?m macroglobulinemia receiving bortezomib-based treatment from December 2008 to October 2015 .Three therapeutic regimens included BD (bortezomib and dexamethasone) in one case ,RBD (bortezomib ,rituximab and dexamethasone) in three cases and BCD (bortezomib ,dexamethasone and cyclophosphamide) in eleven cases .Responses ,adverse reactions and survival analysis were evaluated respectively .Results The overall response rate and major response rate were 93 .3% and 80% inclu-ding CR 1 case ,VGPR 2 cases ,PR 9 cases and MR 2 cases .The common adverse events included gastrointestinal (53 .3% ) , leukopenia (20% ) ,infection (20% ) and peripheral neuropathy (26 .7% ) .After a median follow-up of 21 (3-85) months ,the median PFS (progression-free survival) time was 21 (3-36) months and 1 year PFS rate was 83 .3% .Survival analysis showed that two prognostic risk factors related to PFS were high-risk group based on international prognostic scoring system for WM (IPSSWM )(P=0 .015) and the low response to treatment (< PR) (P=0 .024) .Conclusion Bortezomib-based therapeutic regimensexhibited significant efficacyfor patients with WM .IPSSWM and the responses to treatment can be usedto monitor the disease progression and evaluate the therapeutic result .

A Case of Glomerulonephritis in Waldenstr m's Macroglobulinemia(WM) Characterized by Hyaline Thrombi in Glomerulocapillary Lumen / 대한신장학회잡지

Waldenstr m's macroglobulinemia(WM) is a disorder of malignant proliferation of plasmacyoid lymphocytes and monoclonal immunoglobulin M. The renal complications of WM are less common and severe than that of multiple myeloma. We present a case of WM which involved the kidney as massive proteinuria, hematuria and renal failure. A biopy specimen of the kidney reveals the intraglomerular hyaline thrombi of immunoglobulin M paraprotein. Three cycles of plasmapheresis and systemic chemotherapy with chlorambucil and prednisolone show improvement in his renal manifestations. We suppose the glomerulonephritis caused by hyaline thrombi in WM may be reversible, at least in its early stage.

A Case of Waldenstrom's Macroglobulinemia Presented as a Compression Fracture of Spine / 대한혈액학회지

Waldenstrom's macroglobulinemia is a rare disease of plasmacytoid lymphocyte proliferation usually presented without bone lesion which is the common presenting symptom in multiple myeloma. We report a 50-year-old female with Waldenstrom's macroglobulinemia presented as a bony lesion without many other features common in this diesease. She was admitted with the chief complaint of low back pain and low extremity paresthesia for two months. Bone marrow biopsy and aspiration, protein and immune electrophoresis showed findings consistent with Waldenstr m's macroglobulinemia. Magnetic resonance imaging of thoracic spine showed pathologic compression fracture in T6 and T7 with posterior epidural mass at T6 to T7 level. We report this unusual case of Waldenstrom's macroglobulinemia presented as compression fracture of thoracic spine with a review of literatures.

A Case of Pleural Involvement in Patient with Waldenstr m's Macroglobulinemia / 대한혈액학회지

Waldenstr m's macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by lymphocytic tumor infiltration of the bone marrow and monoclonal IgM gammopathy. There had been anecdotal reports of pleural involvement in WM. We experienced a case of WM with pleural involvement and reported here for the first time in Korea with review of literature. A 73-year-old male patient was admitted to our hospital due to dizziness and general weakness. Serum protein electrophoresis showed M-peak in the gamma-globulin region, which was revealed as IgM kappa macroglobulin by serum and urine immunoelectrophoresis. He complained headache, visual disturbance and epistaxis associated with hyperviscosity syndrome and plasma filtration and combination chemotherapy was performed immediately. Symptoms and laboratory parameters such as serum IgM level and globulin fraction were markedly improved thereafter. But during the treatment, insidiously progressive exertional dyspnea was developed and the chest X-ray showed bilateral pleural effusion. The pleural fluid contained abundant plasmacytoid lymphocytes with reactive mesothelial cells. His dyspnea was completely resolved with clearing of the radiographic pleural effusion after continued steroid therapy.

A Case of Waldenstrom's Macroglobulinemia with Multiple Punched-out, Osteolytic Lesions / 대한혈액학회지

Waldenstr m's macroglobulinemia (WM) is a chronic lymphoproliferative disorder characterized by the association of serum monoclonal IgM and marrow infiltration by lymphoid population. A 69-year-old man was first seen for evaluation of anemia. He had complained of generalized weakness and exertional dyspnea. On physical examination there was generalized lymphadenopathy, palpable liver and spleen. He did not show signs and symptoms of hyperviscosity. Skull X-ray demonstrated multiple punched-out, osteolytic lesions. The total serum protein concentration was 10.6g/dL, with a fast gamma peak accounting for 48.3% of the total. The peak was identified as an IgM of the kappa chain type by immunoelectrophoresis. Bence-Jones protein (kappa type) was found in the urine. A Sia test gave positive result. The patient's serum contained a pyroglobulin. Serum viscosity was 5.3. The bone marrow aspirate and biopsy showed infiltration by atypical lymphocytes (about 90% of all nucleated cells), plasmacytic lymphocytes, and plasma cells. By flow cytometric analysis, these cells expressed CD19 (54%), CD20 (80.5%), and CD38 (92%), but did not express CD3, CD5, and CD10. Biopsy specimen from cervical lymph node demonstrated proliferation of abnormal lymphoid cells, composed of a similar population of cells seen in the bone marrow. He was treated with cyclophosphamide, vincristine, and prednisolone and now he improves slightly in clinical and laboratory aspects.