Waldenström macroglobulinemia is a rare lymphoid tumor accounting for 2% of all hematological malignancies. Renal complications are less common compared to multiple myeloma, with the most frequent renal manifesta...Waldenström macroglobulinemia is a rare lymphoid tumor accounting for 2% of all hematological malignancies. Renal complications are less common compared to multiple myeloma, with the most frequent renal manifestations being microproteinuria and microhematuria. This paper presents a case of Waldenström macroglobulinemia with acute kidney injury as the initial manifestation. A 75-year-old male was admitted to the Affiliated Hospital of Hebei University after elevated blood creatinine levels were detected for one day. Upon admission, his blood creatinine was 255 μmol/L, urine protein was 1+, urine erythrocytes were negative, electrophoresis showed IgM positivity in the κ-region, and a bone marrow biopsy indicated a tendency towards lymphoplasmacytic lymphoma. The patient was discharged after receiving a treatment regimen of prednisone acetate, thalidomide, and cyclophosphamide, and continued oral medication outside the hospital. The patient returned two weeks later due to diarrhea and was found to have a blood creatinine level of 985 μmol/L, along with severe acidosis and hyperkalemia. The patient refused renal replacement therapy and was not followed up, resulting in a poor prognosis. Additionally, a review of the literature is provided to contextualize this case within the broader scope of existing research.展开更多
Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical charac...Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients,and compared them with the Western reports.Results The median age was 62 years old with a male-to-female ratio of 3.74.The most common symptoms at diagnosis were fatigue （77.8％） and bleeding （20％）,while only 6 patients （6.7％） were asymptomatic.In the univariate analysis,age ＞62 years,thrombocytopenia,leucopenia,cytopenias ≥2,and high risk on the international prognostic scoring system for WM were the adverse risk factors,but only age ＞62 years and ≥2 cytopenias were the independent prognostic factors in the multivariate analysis.Using age ＜62 years and ≥2 cytopenias,three significantly different prognostic groups could been distinguished,with 5-year overall survival of 71.6％,48.6％,and 17.0％ （P ＜0.001）.Conclusion Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.展开更多
BACKGROUND Waldenstrom macroglobulinemia(WM) is a type of small lymphocytic lymphoma that mainly affects the bone marrow, spleen, and lymph nodes. A subset of patients with WM demonstrates extramedullary involvement(4...BACKGROUND Waldenstrom macroglobulinemia(WM) is a type of small lymphocytic lymphoma that mainly affects the bone marrow, spleen, and lymph nodes. A subset of patients with WM demonstrates extramedullary involvement(4.4%),and the most frequent extramedullary disease site involved is the lungs(30%).CASE SUMMARY A 60-year-old male patient who experienced intermittent breath-holding for 6 mo was admitted on August 14, 2017. Chest computed tomography indicated multiple pulmonary cavities in the upper lobes of both lungs, with pulmonary consolidation, ground-glass opacities, patchy infiltrates, fibrous bands, large bullae, and enlarged lymph nodes in the mediastinum. The patient was a heavy smoker(20 cigarettes/d for 40 years). Diagnostic fiberoptic bronchoscopy revealed normal findings. Serological examination revealed a remarkable increase in serum immunoglobulin M levels(30.24 g/L;normal: 0.4-2.30 g/L). A computed tomography-guided percutaneous pulmonary biopsy was performed in the left lower lobe of the lung with pulmonary consolidation and indicated that the alveolar structure disappeared and that a large amount of amyloid-like deposition was present along with the infiltration of very few lymphocytes and plasma cells. The patient was treated with the combined treatment of dexamethasone + rituximab + lenalidomide over four courses. Serum immunoglobulin M did not normalize, and he received ibrutinib +dexamethasone.CONCLUSION This patient with WM and lung amyloidosis had a wide range of pulmonary lesions and a variety of morphological features, which was a rare case. Yet, some changes might be ascribed to heavy smoking.展开更多
BACKGROUND Waldenström macroglobulinemia(WM)is a distinct clinicopathologic entity characterized by the infiltration of the bone marrow by clonal lymphoplasmacytic cells that produce monoclonal immunoglobulin M(I...BACKGROUND Waldenström macroglobulinemia(WM)is a distinct clinicopathologic entity characterized by the infiltration of the bone marrow by clonal lymphoplasmacytic cells that produce monoclonal immunoglobulin M(IgM)in the blood,and patients may present with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood.Funduscopic abnormalities were noted in some of the patients due to hyperviscosity or other retinal lesions.Optical coherence tomography angiography(OCTA)as a noninvasive imaging tool can give qualitative and quantitative information about the status of retinal and choroidal vessels,which might be useful for diagnosing patients with WM-associated retinopathy.CASE SUMMARY The patient was a 67-year-old man who presented with sudden visual disturbance in both eyes.Ophthalmic tests showed that best corrected visual acuity(BCVA)for this patient was 20/100 in the right eye and 20/1000 in the left eye.Fundus examination,optical coherence tomography(OCT),and OCTA revealed substantial bilateral optic disc edema,dilated and tortuous retinal veins,and diffuse intraretinal blot hemorrhages and edema which were consistent with bilateral central retinal vein occlusion(CRVO).Meanwhile,remarkable bilateral serous macular detachments(SMD)were noticed on OCT.Systemic examinations showed that the patient had anemia and extremely high level of monoclonal IgM and infiltration of clonal lymphoplasmacytic cells in bone marrow.The diagnosis of WM with hyperviscosity and retinopathy was made based on the clinical manifestation and laboratory findings.He was subsequently treated with intravitreal ranibizumab injection,plasmapheresis,and bortezomib plus rituximab with dexamethasone.Six months after treatments,the central macular volume decreased by 16.1%in the right eye and 28.6%in the left eye on OCT,and the patient’s BCVA was improved to 20/60 in the right eye and 20/400 in the left eye.Very good partial response was obtained after systemic treatment.CONCLUSION WM may affect visual function and present as bilateral CRVO.OCTA can show characteristic changes in both retina and choroid vasculatures,which might be of great value for diagnosing or following patients with WM retinopathy.Intravitreal anti-vascular endothelial growth factor treatment combined with systemic therapy might be beneficial for WM patients with retinopathy(SMD and CRVO).展开更多
BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who d...BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment.A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years.Her INMZL was confirmed due to left cervical lymphadenopathy.The patient presented with oedema in both lower limbs one year ago,and was diagnosed with secondary light chain amyloidosis.Treatment with the BC regimen(rituximab 375 mg/m~2 monthly for 6-8 courses,and bendamustine 90 mg/m~2 per day × 2,monthly for six courses) was initiated,but not tolerated due to toxic side effects.Bortezomibbased therapy was given for two months,including bortezomib,dexamethasone,and zanubrutinb.Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.展开更多
BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diag...BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diagnoses of diffuse large B-cell lymphoma(DLBCL),acute myeloid leukemia(AML),and untreated lymphoplasmacytic lymphoma/Waldenström macroglobulinemia(LPL/WM)in the same patient have not been reported.Here we report one such case.CASE SUMMARY An 89-year-old man had a chest wall mass histopathologically diagnosed as DLBCL.The bone marrow and peripheral blood contained two groups of cells.One group of cells fulfilled the criteria of AML,and the other revealed the features of small B lymphocytic proliferative disorder,which we considered LPL/WM.Multiple chromosomal or genetic changes were detected in bone marrow mononuclear cells,including ATM deletion,CCND1 amplification,mutations of MYD88(L265P)and TP53,WT1 overexpression,and fusion gene of BIRC2-ARAP1,as well as complex chromosomal abnormalities.The patient refused chemotherapy because of old age and died of pneumonia 1 mo after the final diagnosis.CONCLUSION The coexistence of DLBCL,AML,and untreated LPL/WM in the same patient is extremely rare,which probably results from multiple steps of genetic abnormalities.Asymptomatic LPL/WM might have occurred first,then myelodysplastic syndromerelated AML developed,and finally aggressive DLBCL arose.Therefore,medical staff should pay attention to this rare phenomenon to avoid misdiagnoses.展开更多
Early progression of disease within 24 months(POD24)of diagnosis is associated with inferior overall survival(OS)in follicular lymphoma,but its prognostic role in Waldenström’s macroglobulinemia(WM)is still uncl...Early progression of disease within 24 months(POD24)of diagnosis is associated with inferior overall survival(OS)in follicular lymphoma,but its prognostic role in Waldenström’s macroglobulinemia(WM)is still unclear.Here,we per-formed a retrospective analysis of 373 patients pooled from the database of the Chinese Registration Network for WM(CRNWM)to determine the outcomes of early progressors.POD24 occurred in 98 evaluable patients(26.3%).The median OS in patients with POD24 was 40 months(95%CI:15.2–64.0),which was significantly shorter than that with-out POD24(156 months,95%CI:123.6–188.4;HR:6.587,95%CI 4.392–9.881,P<0.0001).Moreover,subgroup analysis showed that POD24 still maintained its predictive ability of inferior OS in patients treated with rituximab or borte-zomib-based treatment.Patients with a very high-risk rIPSSWM stage were more likely to have POD24(P=0.020).In conclusion,POD24 was associated with poorer survival and may represent a useful endpoint in future prospective clinical trials.展开更多
文摘Waldenström macroglobulinemia is a rare lymphoid tumor accounting for 2% of all hematological malignancies. Renal complications are less common compared to multiple myeloma, with the most frequent renal manifestations being microproteinuria and microhematuria. This paper presents a case of Waldenström macroglobulinemia with acute kidney injury as the initial manifestation. A 75-year-old male was admitted to the Affiliated Hospital of Hebei University after elevated blood creatinine levels were detected for one day. Upon admission, his blood creatinine was 255 μmol/L, urine protein was 1+, urine erythrocytes were negative, electrophoresis showed IgM positivity in the κ-region, and a bone marrow biopsy indicated a tendency towards lymphoplasmacytic lymphoma. The patient was discharged after receiving a treatment regimen of prednisone acetate, thalidomide, and cyclophosphamide, and continued oral medication outside the hospital. The patient returned two weeks later due to diarrhea and was found to have a blood creatinine level of 985 μmol/L, along with severe acidosis and hyperkalemia. The patient refused renal replacement therapy and was not followed up, resulting in a poor prognosis. Additionally, a review of the literature is provided to contextualize this case within the broader scope of existing research.
基金This work was supported by grants from Science and Technology Infrastructure Program of Tianjin （No.12ZCDZSY17600）,National Public Health Grand Research Foundation （No.201202017）,and National Nature Science Foundation of China （No.81370632 and No.81200395）.
文摘Background Waldenstr（o）m macroglobulinemia （WM） is an uncommon lymphoid malignancy.The characteristics and prognosis of WM have never been systematically studied in the East.Methods We analyzed the clinical characteristics and the prognostic factors of 90 Chinese WM patients,and compared them with the Western reports.Results The median age was 62 years old with a male-to-female ratio of 3.74.The most common symptoms at diagnosis were fatigue （77.8％） and bleeding （20％）,while only 6 patients （6.7％） were asymptomatic.In the univariate analysis,age ＞62 years,thrombocytopenia,leucopenia,cytopenias ≥2,and high risk on the international prognostic scoring system for WM were the adverse risk factors,but only age ＞62 years and ≥2 cytopenias were the independent prognostic factors in the multivariate analysis.Using age ＜62 years and ≥2 cytopenias,three significantly different prognostic groups could been distinguished,with 5-year overall survival of 71.6％,48.6％,and 17.0％ （P ＜0.001）.Conclusion Distinct characteristics exist in WM in China compared to the West and we describe a new simple prognostic model for newly diagnosed WM patients.
文摘BACKGROUND Waldenstrom macroglobulinemia(WM) is a type of small lymphocytic lymphoma that mainly affects the bone marrow, spleen, and lymph nodes. A subset of patients with WM demonstrates extramedullary involvement(4.4%),and the most frequent extramedullary disease site involved is the lungs(30%).CASE SUMMARY A 60-year-old male patient who experienced intermittent breath-holding for 6 mo was admitted on August 14, 2017. Chest computed tomography indicated multiple pulmonary cavities in the upper lobes of both lungs, with pulmonary consolidation, ground-glass opacities, patchy infiltrates, fibrous bands, large bullae, and enlarged lymph nodes in the mediastinum. The patient was a heavy smoker(20 cigarettes/d for 40 years). Diagnostic fiberoptic bronchoscopy revealed normal findings. Serological examination revealed a remarkable increase in serum immunoglobulin M levels(30.24 g/L;normal: 0.4-2.30 g/L). A computed tomography-guided percutaneous pulmonary biopsy was performed in the left lower lobe of the lung with pulmonary consolidation and indicated that the alveolar structure disappeared and that a large amount of amyloid-like deposition was present along with the infiltration of very few lymphocytes and plasma cells. The patient was treated with the combined treatment of dexamethasone + rituximab + lenalidomide over four courses. Serum immunoglobulin M did not normalize, and he received ibrutinib +dexamethasone.CONCLUSION This patient with WM and lung amyloidosis had a wide range of pulmonary lesions and a variety of morphological features, which was a rare case. Yet, some changes might be ascribed to heavy smoking.
基金Supported by the National Youth Top-notch Talent of Ten Thousand Talent Program,No.89602300.
文摘BACKGROUND Waldenström macroglobulinemia(WM)is a distinct clinicopathologic entity characterized by the infiltration of the bone marrow by clonal lymphoplasmacytic cells that produce monoclonal immunoglobulin M(IgM)in the blood,and patients may present with symptoms related to the infiltration of the hematopoietic tissues or the effects of monoclonal IgM in the blood.Funduscopic abnormalities were noted in some of the patients due to hyperviscosity or other retinal lesions.Optical coherence tomography angiography(OCTA)as a noninvasive imaging tool can give qualitative and quantitative information about the status of retinal and choroidal vessels,which might be useful for diagnosing patients with WM-associated retinopathy.CASE SUMMARY The patient was a 67-year-old man who presented with sudden visual disturbance in both eyes.Ophthalmic tests showed that best corrected visual acuity(BCVA)for this patient was 20/100 in the right eye and 20/1000 in the left eye.Fundus examination,optical coherence tomography(OCT),and OCTA revealed substantial bilateral optic disc edema,dilated and tortuous retinal veins,and diffuse intraretinal blot hemorrhages and edema which were consistent with bilateral central retinal vein occlusion(CRVO).Meanwhile,remarkable bilateral serous macular detachments(SMD)were noticed on OCT.Systemic examinations showed that the patient had anemia and extremely high level of monoclonal IgM and infiltration of clonal lymphoplasmacytic cells in bone marrow.The diagnosis of WM with hyperviscosity and retinopathy was made based on the clinical manifestation and laboratory findings.He was subsequently treated with intravitreal ranibizumab injection,plasmapheresis,and bortezomib plus rituximab with dexamethasone.Six months after treatments,the central macular volume decreased by 16.1%in the right eye and 28.6%in the left eye on OCT,and the patient’s BCVA was improved to 20/60 in the right eye and 20/400 in the left eye.Very good partial response was obtained after systemic treatment.CONCLUSION WM may affect visual function and present as bilateral CRVO.OCTA can show characteristic changes in both retina and choroid vasculatures,which might be of great value for diagnosing or following patients with WM retinopathy.Intravitreal anti-vascular endothelial growth factor treatment combined with systemic therapy might be beneficial for WM patients with retinopathy(SMD and CRVO).
基金Supported by Medical and Health Research Project of Hainan Province,No. 21A200197。
文摘BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment.A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years.Her INMZL was confirmed due to left cervical lymphadenopathy.The patient presented with oedema in both lower limbs one year ago,and was diagnosed with secondary light chain amyloidosis.Treatment with the BC regimen(rituximab 375 mg/m~2 monthly for 6-8 courses,and bendamustine 90 mg/m~2 per day × 2,monthly for six courses) was initiated,but not tolerated due to toxic side effects.Bortezomibbased therapy was given for two months,including bortezomib,dexamethasone,and zanubrutinb.Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.
基金Supported by the National Natural Science Foundation of China,No.81700130Nanjing Medical University Science and Technology Development Fund.
文摘BACKGROUND The Coexistence of myeloid and lymphoid malignancies is rare.Myeloid leukemia occurs more frequently as a secondary event in patients receiving chemotherapy agents for lymphoid malignancies.Synchronous diagnoses of diffuse large B-cell lymphoma(DLBCL),acute myeloid leukemia(AML),and untreated lymphoplasmacytic lymphoma/Waldenström macroglobulinemia(LPL/WM)in the same patient have not been reported.Here we report one such case.CASE SUMMARY An 89-year-old man had a chest wall mass histopathologically diagnosed as DLBCL.The bone marrow and peripheral blood contained two groups of cells.One group of cells fulfilled the criteria of AML,and the other revealed the features of small B lymphocytic proliferative disorder,which we considered LPL/WM.Multiple chromosomal or genetic changes were detected in bone marrow mononuclear cells,including ATM deletion,CCND1 amplification,mutations of MYD88(L265P)and TP53,WT1 overexpression,and fusion gene of BIRC2-ARAP1,as well as complex chromosomal abnormalities.The patient refused chemotherapy because of old age and died of pneumonia 1 mo after the final diagnosis.CONCLUSION The coexistence of DLBCL,AML,and untreated LPL/WM in the same patient is extremely rare,which probably results from multiple steps of genetic abnormalities.Asymptomatic LPL/WM might have occurred first,then myelodysplastic syndromerelated AML developed,and finally aggressive DLBCL arose.Therefore,medical staff should pay attention to this rare phenomenon to avoid misdiagnoses.
基金supported by grants from the National Nature Science Foundation of China(82370197,81900203,81970187,82170193 and 81920108006)Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences(2022-I2M-1-022 and 2021-I2M-C&T-B-081).
文摘Early progression of disease within 24 months(POD24)of diagnosis is associated with inferior overall survival(OS)in follicular lymphoma,but its prognostic role in Waldenström’s macroglobulinemia(WM)is still unclear.Here,we per-formed a retrospective analysis of 373 patients pooled from the database of the Chinese Registration Network for WM(CRNWM)to determine the outcomes of early progressors.POD24 occurred in 98 evaluable patients(26.3%).The median OS in patients with POD24 was 40 months(95%CI:15.2–64.0),which was significantly shorter than that with-out POD24(156 months,95%CI:123.6–188.4;HR:6.587,95%CI 4.392–9.881,P<0.0001).Moreover,subgroup analysis showed that POD24 still maintained its predictive ability of inferior OS in patients treated with rituximab or borte-zomib-based treatment.Patients with a very high-risk rIPSSWM stage were more likely to have POD24(P=0.020).In conclusion,POD24 was associated with poorer survival and may represent a useful endpoint in future prospective clinical trials.
