BACKGROUND Metastatic neuroendocrine carcinoma(NEC) of bone marrow is uncommon.Here,we report a case of bone marrow metastatic NEC with an unknown primary site.CASE SUMMARY A 73-year-old Chinese woman was admitted to ...BACKGROUND Metastatic neuroendocrine carcinoma(NEC) of bone marrow is uncommon.Here,we report a case of bone marrow metastatic NEC with an unknown primary site.CASE SUMMARY A 73-year-old Chinese woman was admitted to our hospital because marked chest distress and asthma lasting 1 d on March 18,2018.She was initially diagnosed with pulmonary infection,cardiac insufficiency,thrombocytopenia and severe anemia.Following treatment with antibiotic therapy,diuresis and blood transfusion,the patient’s symptoms greatly improved.After bone marrow examinations,the patient was diagnosed with bone marrow metastatic NEC,bone marrow necrosis(BMN) and secondary myelofibrosis(MF).Further imaging workup did not show the primary tumor,we presumed that the primary site might regress spontaneously or merely be unexplored due to lack of positron emission tomography with gallium peptide.Everolimus(10 mg/d) was added to the treatment and the best supportive and symptomatic therapies were also administered.Unfortunately,the patient’s condition continued to deteriorate and she died on May 15,2018.CONCLUSION Bone marrow invasion of NEC is rare and our patient who suffered from bone marrow metastatic NEC as well as secondary BMN and MF had an extremely poor prognosis.Bone marrow biopsy plays an important role in the diagnosis of solid tumors invading bone marrow.展开更多
Factors associated with complete and durable remissions after anti-CD19 chimeric antigen receptor T(CAR-T)cell immunotherapy for relapsed or refractory non-Hodgkin lymphoma(r/r NHL)have not been well characterized.In ...Factors associated with complete and durable remissions after anti-CD19 chimeric antigen receptor T(CAR-T)cell immunotherapy for relapsed or refractory non-Hodgkin lymphoma(r/r NHL)have not been well characterized.In this study,we found that the different sites of extranodal involvement may affect response,overall survival(OS),and progression-free survival(PFS)in patients with r/r NHL treated with anti-CD19 CAR-T cells.In a cohort of 32 treated patients,12(37.5%)and 8(25%)patients exhibited soft tissue lymphoma and bone marrow(BM)infiltrations,respectively,and 13(41%)patients exhibited infiltration at other sites.The factors that may affect prognosis were identified through multivariable analysis.As an independent risk factor,soft tissue infiltration was the only factor significantly correlated with adverse prognosis(P<0.05),whereas other factors did not reach statistical significance.Furthermore,the site of extranodal tumor infiltration significantly and negatively affected OS and PFS in patients with r/r NHL treated with anti-CD19 CAR-T cell therapy.PFS and OS in patients with BM involvement were not significantly different from those of patients with lymph node involvement alone.Thus,anti-CD19 CAR-T cell therapy may improve the prognosis of patients with BM infiltration.展开更多
The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML fro...The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.展开更多
The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced p...The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.展开更多
目的观察芪胶升白胶囊在鼻咽癌患者同步放化疗所致骨髓抑制中的治疗作用。方法 90例同步放化疗过程中出现骨髓抑制的局部晚期鼻咽癌患者随机分为3组,实验组:芪胶升白胶囊+重组人粒细胞集落刺激因子(recombinant human granulocyte colon...目的观察芪胶升白胶囊在鼻咽癌患者同步放化疗所致骨髓抑制中的治疗作用。方法 90例同步放化疗过程中出现骨髓抑制的局部晚期鼻咽癌患者随机分为3组,实验组:芪胶升白胶囊+重组人粒细胞集落刺激因子(recombinant human granulocyte colony-stimulating factor,G-CSF);对照1组:利血生+G-CSF;对照2组:单独使用G-CSF,治疗同步放化疗所致骨髓抑制,比较其疗效及不良反应。结果治疗结束时,实验组与对照1、2组在骨髓保护方面,差异有统计学意义(P<0.05);同步放化疗结束后,及结束后1、2周,实验组较对照1、2组骨髓抑制发生率明显降低,差异均有统计学意义(P<0.05),且无不良反应。结论芪胶升白胶囊对于同步放化疗所致的白细胞下降治疗作用显著,同时可以起到较好的骨髓保护作用,且无明显不良反应,价格低廉,可作为放化疗骨髓抑制时的常规口服用药。展开更多
文摘BACKGROUND Metastatic neuroendocrine carcinoma(NEC) of bone marrow is uncommon.Here,we report a case of bone marrow metastatic NEC with an unknown primary site.CASE SUMMARY A 73-year-old Chinese woman was admitted to our hospital because marked chest distress and asthma lasting 1 d on March 18,2018.She was initially diagnosed with pulmonary infection,cardiac insufficiency,thrombocytopenia and severe anemia.Following treatment with antibiotic therapy,diuresis and blood transfusion,the patient’s symptoms greatly improved.After bone marrow examinations,the patient was diagnosed with bone marrow metastatic NEC,bone marrow necrosis(BMN) and secondary myelofibrosis(MF).Further imaging workup did not show the primary tumor,we presumed that the primary site might regress spontaneously or merely be unexplored due to lack of positron emission tomography with gallium peptide.Everolimus(10 mg/d) was added to the treatment and the best supportive and symptomatic therapies were also administered.Unfortunately,the patient’s condition continued to deteriorate and she died on May 15,2018.CONCLUSION Bone marrow invasion of NEC is rare and our patient who suffered from bone marrow metastatic NEC as well as secondary BMN and MF had an extremely poor prognosis.Bone marrow biopsy plays an important role in the diagnosis of solid tumors invading bone marrow.
基金This study was supported by grants from the Municipal Hospital Frontier Technology Joint Project of Shanghai City(No.SHDC12015108)the National Natural Science Foundation of China(Nos.81830004 and 31301118).
文摘Factors associated with complete and durable remissions after anti-CD19 chimeric antigen receptor T(CAR-T)cell immunotherapy for relapsed or refractory non-Hodgkin lymphoma(r/r NHL)have not been well characterized.In this study,we found that the different sites of extranodal involvement may affect response,overall survival(OS),and progression-free survival(PFS)in patients with r/r NHL treated with anti-CD19 CAR-T cells.In a cohort of 32 treated patients,12(37.5%)and 8(25%)patients exhibited soft tissue lymphoma and bone marrow(BM)infiltrations,respectively,and 13(41%)patients exhibited infiltration at other sites.The factors that may affect prognosis were identified through multivariable analysis.As an independent risk factor,soft tissue infiltration was the only factor significantly correlated with adverse prognosis(P<0.05),whereas other factors did not reach statistical significance.Furthermore,the site of extranodal tumor infiltration significantly and negatively affected OS and PFS in patients with r/r NHL treated with anti-CD19 CAR-T cell therapy.PFS and OS in patients with BM involvement were not significantly different from those of patients with lymph node involvement alone.Thus,anti-CD19 CAR-T cell therapy may improve the prognosis of patients with BM infiltration.
文摘The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.
文摘The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.
