No abstract available.
Drug Therapy* ; Waldenstrom Macroglobulinemia*

Objective: To investigate the efficacy and safety of Bruton tyrosine kinase inhibitors (BTKi) ibrutinib or zanubrutinib monotherapy in newly diagnosed patients with Waldenström macroglobulinemia (WM) . Methods: The efficacy and adverse effects of 58 patients with newly diagnosed WM receiving BTKi monotherapy in Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine were analyzed retrospectively from January 2018 to August 2022. Results: The response of 55 patients may be examined. Forty patients received ibrutinib monotherapy for a median of 15 months, with an overall response rate (ORR) of 85%, a main remission rate (MRR) of 70%, and a very good partial remission (VGPR) rate of 10%. Fifteen patients received zanubrutinib monotherapy for a median of 13 months, with an ORR of 93%, an MRR of 73%, and a VGPR rate of 0%. For various reasons, 10 patients were converted from ibrutinib to zanubrutinib. Ibrutinib treatment lasted an average of 7.5 months before conversion. The median duration of zanubrutinib therapy after conversion was 3.5 months. The ORRs before and after conversion were 90% and 100%, MRRs were 80% and 80%, and VGPR rates were 10% and 50%, respectively. After a median of 16 months, the 24-month progression-free survival (PFS) rate of patients who received both BTKi was 86%. PFS did not differ statistically across individuals with low, medium, and high-risk ISS scores (P=0.998). All of the patients survived. The most common side effects of BTKi were neutropenia and thrombocytopenia, which occurred in 12% and 10% of all patients, respectively. Ibrutinib accounts for 5% of atrial fibrillation, and zanubrutinib has a 7% risk of bleeding. Conclusions: In treating WM, ibrutinib or zanubrutinib provides good efficacy and tolerable adverse effects.
Humans ; China ; Retrospective Studies ; Treatment Outcome ; Tyrosine Protein Kinase Inhibitors/therapeutic use* ; Waldenstrom Macroglobulinemia/drug therapy*

Aged ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Male ; Retinal Detachment ; complications ; drug therapy ; Waldenstrom Macroglobulinemia ; complications ; drug therapy

PURPOSE: The authors report a case of bilateral simultaneous central retinal vein occlusion caused by Waldenstrom's macroglobulinemia. CASE SUMMARY: A 65-year-old man presented to our department complaining of decreased visual acuity for the duration of about 6 months. On his initial visit, best-corrected visual acuity was 0.02 in the right eye and 0.06 in the left eye. Based on the findings of a funduscopic examination, the patient had bilateral diffuse retinal hemorrhages, dilated tortuous veins, and macular edema. He had experienced recurrent spontaneous epistaxis 6 months previously and had undergone treatments such as intravitreal bevacizumab injection and intravitreal dexamethasone implantation at another hospital. Laboratory tests at that hospital showed anemia and hyperproteinemia, for which he was referred to our hemato-oncology department. Bone marrow biopsy was consistent with Waldenstrom's macroglobulinemia/lymphoplasmacytoid lymphoma, and he was treated with systemic chemotherapy. One year after the systemic chemotherapy, his best-corrected visual acuity was 0.15 in the right eye and 0.6 in the left eye. Funduscopy showed decreased bilateral retinal hemorrhages and macular edema. CONCLUSIONS: When simultaneous bilateral central retinal vein occlusion occurs in a patient with no other underlying disease such as hypertension or diabetes, it might be a sign of serum hyperviscosity, and there should be a very high level of suspicion for presence or progression of systemic disease. If such a disease is properly and timely diagnosed, effective early systemic evaluation and therapy can be administered, and it is important to have initial general treatment as well as ophthalmic treatment.
Aged ; Anemia ; Bevacizumab ; Biopsy ; Bone Marrow ; Dexamethasone ; Drug Therapy ; Epistaxis ; Humans ; Hypertension ; Lymphoma ; Macular Edema ; Retinal Hemorrhage ; Retinal Vein* ; Veins ; Visual Acuity ; Waldenstrom Macroglobulinemia*

OBJECTIVE To study the morphology, immunology, cyto- and molecular genetics of a patient with lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM), deletion of P53 gene and rearrangement of clonal T cell receptors-delta (TCR-delta) gene. METHODS The cell morphology and immunocytochemistry were analyzed by bone marrow testing and biopsy. Cellular immunology was analyzed by flow cytometry. Genetic analysis was carried out by chromosome karyotyping, fluorescent in situ hybridization (FISH) and polymerase chain reaction (PCR). Immunoglobulin M (IgM) in serum and urine was assayed by immunofixation electrophoresis. And the effect of chlorambucil therapy was evaluated. RESULTS Bone marrow biopsy suggested that the patient was of B lymphocyte type and had abnormal increase of lymphocytoid plasma cells, which were CD38 and CD138 positive. The patient had a normal male karyotype. FISH and PCR analysis of peripheral blood samples suggested deletion of P53 gene and rearrangement of TCR-delta gene. Immunofixation electrophoresis has detected IgM-kappa in both serum and urine. The patient showed partial response to chlorambucil. CONCLUSION In addition to typical clinical features, bone marrow examination, flow cytometry, histochemistry and immunophenotyping, testing for P53 gene deletion and lymphocyte gene rearrangement can facilitate the diagnosis and treatment of LPL/WM.
Aged ; Gene Rearrangement, delta-Chain T-Cell Antigen Receptor ; Genes, p53 ; Humans ; In Situ Hybridization, Fluorescence ; Male ; Polymerase Chain Reaction ; Waldenstrom Macroglobulinemia ; drug therapy ; genetics

Antibodies, Monoclonal, Murine-Derived ; adverse effects ; Antineoplastic Agents ; adverse effects ; Humans ; Lung Diseases, Interstitial ; chemically induced ; Rituximab ; Waldenstrom Macroglobulinemia ; drug therapy

The treatment outcomes with conventional second-line chemotherapy or radiotherapy aregenerally very poor for patients with relapsed primary CNS lymphoma (PCNSL). We treated three relapsed PCNSL patients with high-dose cytarabine plus etoposide (CYVE) chemotherapy, and this was followed by autologous stem cell transplantation (ASCT). The salvage CYVE chemotherapy consisted of cytarabine 2g/m2/d on days 2 to 5 in a 3-hour infusion and 50mg/m2/d on days 1 to 5 in a 12-hourinfusion, and etoposide 200mg/m2/d on days 2 to 5 in a 2-hour infusion. After two cycles of CYVE chemotherapy, two patients achieved a complete response (CR), and one patient achieved a partial response (PR). All three patients experienced febrile neutropenia and grade 4 thrombocytopenia with the CYVE chemotherapy. However, the hematologic toxicities were well managed without any complications. The conditioning regimen for ASCT consisted of BCNU 300mg/m2 on day -7, etoposide 100mg/m2 on days -6 to -3, cytarabine 100mg/m2 on days -6 to -3, and cyclophosphamide 35mg/kg on days -6 to -3 (BEAC). After ASCT, the patient who initially showed a PR with CYVE chemotherapy then achieved a CR. At the time of this report, one patient remained alive in CR for 41 months after CYVE chemotherapy. The remaining two patients experienced relapse 5 months and 4 months after ASCT, respectively, and they ultimately died of disease progression 18 months and 8 months after ASCT, respectively. In our cases, the CYVE chemotherapy+ASCT was well tolerated, and this induced the complete disappearance of the tumor, and one patient showed prolonged disease-free survival. CYVE chemotherapy+ASCT could be a treatment option for relapsed PCNSL.
Anemia, Hemolytic, Autoimmune ; Carmustine ; Cyclophosphamide ; Cytarabine* ; Disease Progression ; Disease-Free Survival ; Drug Therapy ; Etoposide* ; Febrile Neutropenia ; Humans ; Lymphoma* ; Radiotherapy ; Recurrence ; Stem Cell Transplantation* ; Stem Cells* ; Thrombocytopenia ; Waldenstrom Macroglobulinemia

Waldenstrom's macroglobulinemia is a low- grade lymphoproliferative disorder with monoclonal IgM protein. It is characterized by normocytic, normochromic anemia and lymphoplasmacytic marrow infiltration. Chemotherapy with alkylating agents and steroids has been the standard therapy for patients with symptomatic macroglobulinemia. The purine nucleoside analogues, either alone or in combination with other chemotherapeutic agents are increasingly used, and approximately 40% of patients who have received prior therapy with alkylating agents responded. We experienced a case of Waldenstrom's macroglobulinemia suc-cessfully treated with three courses of cladribine, who had previously received unsuccessful therapy using an alkylating agent, steroid and plasmapheresis. Treatment was well tolerated except for frequent upper respiratory infections with severe pancytopenia. A marked and sustained bone marrow suppression occurred in this patient but resolved in three months without any severe infection.
Alkylating Agents ; Anemia ; Bone Marrow ; Cladribine* ; Drug Therapy ; Humans ; Immunoglobulin M ; Lymphoproliferative Disorders ; Pancytopenia ; Plasmapheresis ; Respiratory Tract Infections ; Steroids ; Waldenstrom Macroglobulinemia*

OBJECTIVETo study the morphologic features, immunophenotype, differential diagnosis and prognosis of nodal marginal zone B-cell lymphoma (NMZL).

METHODSLight microscopic examination and immunohistochemical study were carried out in 10 cases of NMZL. Seven of which had follow-up information available.

RESULTSAll cases presented with good performance status at the time of diagnosis. Amongst the 7 cases with follow-up information available, most (6/7) were in advanced clinical stage (stage II to III). The one-year survival rate was 67%. A vaguely nodular growth pattern was observed in most cases of NMZL (5/10). The lymphoma was composed predominantly of atypical lymphoid cells resembling centrocytes (7/10). A predominance of monocytoid B-cell (2/10) or small lymphocytic (1/10) morphology was rare. Instead, the presence of a minor component of monocytoid B cells was not uncommon (5/10). Plasmacytoid or plasmacytic cells were also frequently found (8/10). The proliferation index ranged from 5% to 50%. Follicular dendritic cells appeared atrophic in 7 cases and variably hyperplasic in 3 cases.

CONCLUSIONSPrimary NMZL is rare. It has a unique growth pattern and most cases are composed predominantly of cells resembling centrocytes. Differential diagnosis includes lymphoplasmacytic lymphoma, lymph node involvement by extranodal marginal zone B-cell lymphoma and T-zone hyperplasia. The clinical stage is often high at presentation, with systemic dissemination. The prognosis of NMZL is thus relatively poor.

Adult ; Aged ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Nodes ; pathology ; Lymphoma, B-Cell, Marginal Zone ; drug therapy ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Remission Induction ; Survival Rate ; Waldenstrom Macroglobulinemia ; pathology

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.
Aged ; Bone Marrow ; Dizziness ; Drug Therapy, Combination ; Dyspnea ; Electrophoresis ; Epistaxis ; Filtration ; gamma-Globulins ; Headache ; Humans ; Immunoelectrophoresis ; Immunoglobulin M ; Korea ; Lymphocytes ; Lymphoproliferative Disorders ; Male ; Plasma ; Pleural Effusion ; Thorax ; Waldenstrom Macroglobulinemia*