BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias...BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias in the same patient is an extremely rare incident and has been reported in several cases in the literature.CASE SUMMARY In the present report,we described a rare case of the co-occurrence of CML and plasma cell dyscrasias in a 48-year-old man,and we discussed the reason why monoclonal gammopathy of undetermined significance progressed to smoldering multiple myeloma and eventually to multiple myeloma while being treated with dasatinib for CML.The tyrosine kinase inhibitor treatment and cytogenetic change may contribute to this phenomenon,and clonal hematopoiesis of indeterminate potential may lead to both CML and multiple myeloma cells in a patient.Future studies are warranted to further explain the hidden reasons.CONCLUSION This case highlights that gene translocation may contribute to initiation and sustainability of clonal proliferation.Moreover,the treatment with tyrosine kinase inhibitor and cytogenetic change may contribute to progression from monoclonal gammopathy of undetermined significance to smoldering multiple myeloma and eventually to multiple myeloma.展开更多
BACKGROUND Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils.Gastrointestinal involvement in systemic amyloidosis is common,but is often sub...BACKGROUND Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils.Gastrointestinal involvement in systemic amyloidosis is common,but is often subclinical or presents as vague and nonspecific symptoms.It is rare for gastrointestinal symptoms to be the main presenting symptom in patients with systemic amyloidosis,causing it to be undiagnosed until late-stage disease.CASE SUMMARY A 53 year-old man with diarrhea,hematochezia,and weight loss presented to a community hospital.Colonoscopy with biopsy at that time was suspicious for Crohn disease.Due to worsening symptoms including nausea,vomiting,and a new petechial rash,an abdominal fat pad biopsy was done.The biopsy showed papillary and adnexal dermal amyloid deposition,in a pattern usually seen with cutaneous amyloidosis.However,Cytokeratin 5/6 was negative,excluding cutaneous amyloidosis.The patterns of nodular amyloidosis,subcutaneous amyloid deposits and perivascular amyloid were not seen.Periodic Acid-Schiff stain was negative for lipoid proteinosis,Congo red was positive for apple green birefringence on polarization and amyloid typing confirmed amyloid light chain amyloidosis.Repeat endoscopic biopsies of the gastrointestinal tract showed amyloid deposition from the esophagus to the rectum,in a pattern usually seen in serum amyloid A in the setting of chronic inflammatory diseases,including severe inflammatory bowel disease.Bone marrow biopsy showed kappa-restricted plasma cell neoplasm.CONCLUSION Described is an unusual presentation of primary systemic amyloidosis,highlighting the risk of misdiagnosis with subsequent significant organ dysfunction and high mortality.展开更多
文摘BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias in the same patient is an extremely rare incident and has been reported in several cases in the literature.CASE SUMMARY In the present report,we described a rare case of the co-occurrence of CML and plasma cell dyscrasias in a 48-year-old man,and we discussed the reason why monoclonal gammopathy of undetermined significance progressed to smoldering multiple myeloma and eventually to multiple myeloma while being treated with dasatinib for CML.The tyrosine kinase inhibitor treatment and cytogenetic change may contribute to this phenomenon,and clonal hematopoiesis of indeterminate potential may lead to both CML and multiple myeloma cells in a patient.Future studies are warranted to further explain the hidden reasons.CONCLUSION This case highlights that gene translocation may contribute to initiation and sustainability of clonal proliferation.Moreover,the treatment with tyrosine kinase inhibitor and cytogenetic change may contribute to progression from monoclonal gammopathy of undetermined significance to smoldering multiple myeloma and eventually to multiple myeloma.
文摘BACKGROUND Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils.Gastrointestinal involvement in systemic amyloidosis is common,but is often subclinical or presents as vague and nonspecific symptoms.It is rare for gastrointestinal symptoms to be the main presenting symptom in patients with systemic amyloidosis,causing it to be undiagnosed until late-stage disease.CASE SUMMARY A 53 year-old man with diarrhea,hematochezia,and weight loss presented to a community hospital.Colonoscopy with biopsy at that time was suspicious for Crohn disease.Due to worsening symptoms including nausea,vomiting,and a new petechial rash,an abdominal fat pad biopsy was done.The biopsy showed papillary and adnexal dermal amyloid deposition,in a pattern usually seen with cutaneous amyloidosis.However,Cytokeratin 5/6 was negative,excluding cutaneous amyloidosis.The patterns of nodular amyloidosis,subcutaneous amyloid deposits and perivascular amyloid were not seen.Periodic Acid-Schiff stain was negative for lipoid proteinosis,Congo red was positive for apple green birefringence on polarization and amyloid typing confirmed amyloid light chain amyloidosis.Repeat endoscopic biopsies of the gastrointestinal tract showed amyloid deposition from the esophagus to the rectum,in a pattern usually seen in serum amyloid A in the setting of chronic inflammatory diseases,including severe inflammatory bowel disease.Bone marrow biopsy showed kappa-restricted plasma cell neoplasm.CONCLUSION Described is an unusual presentation of primary systemic amyloidosis,highlighting the risk of misdiagnosis with subsequent significant organ dysfunction and high mortality.
