BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-Europ...BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.CASE SUMMARY The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body.Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers,sparing the epidermis.Immunohistochemical staining revealed CD20,cyclin-D1,CD5,and SOX-11 expression.Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement.Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved;these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease.In this case,positron emission tomography scanning and clinical staging revealed no systemic involvement,and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease.The prognosis of primary cutaneous MCL is relatively good.Our patient received six cycles of chemotherapy,and the cutaneous manifestations presented almost complete remission.CONCLUSION Primary cutaneous MCL is rare,and its prognosis is relatively favorable.However,correct diagnosis is a prerequisite for proper treatment.展开更多
BACKGROUND QT interval prolongation can induce torsades de pointes(TdP),a potentially fatal ventricular arrhythmia.Recently,an increasing number of non-cardiac drugs have been found to cause QT prolongation and/or TdP...BACKGROUND QT interval prolongation can induce torsades de pointes(TdP),a potentially fatal ventricular arrhythmia.Recently,an increasing number of non-cardiac drugs have been found to cause QT prolongation and/or TdP onset.Moreover,recent findings have demonstrated the key roles of systemic inflammatory activation and fever in promoting long-QT syndrome(LQTS)and TdP development.CASE SUMMARY A 30-year-old woman was admitted with a moderate to high-grade episodic fever for two weeks.The patient was administered with multiple antibiotics after hospitalization but still had repeating fever and markedly elevated C-reactive protein.Once after a high fever,the patient suddenly lost consciousness,and electrocardiogram(ECG)showed transient TdP onset after frequent premature ventricular contraction.The patient recovered sinus rhythm and consciousness spontaneously,and post-TdP ECG revealed a prolonged QTc interval of 560 ms.The patient’s clinical manifestations and unresponsiveness to the antibiotics led to the final diagnosis of adult-onset Still’s disease(AOSD).There was no evidence of cardiac involvement.After the AOSD diagnosis,discontinuation of antibiotics and immediate initiation of intravenous dexamethasone administration resulted in the normal temperature and QTc interval.The genetic analysis identified that the patient and her father had heterozygous mutations in KCNH2(c.1370C>T)and AKAP9(c.7725A>C).During the 2-year follow-up period,the patient had no recurrence of any arrhythmia and maintained normal QTc interval.CONCLUSION This case study highlights the risk of systemic inflammatory activation and antibiotic-induced TdP/LQTS onset.Genetic analysis should be considered to identify individuals at high risk of developing TdP.展开更多
文摘BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.CASE SUMMARY The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body.Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers,sparing the epidermis.Immunohistochemical staining revealed CD20,cyclin-D1,CD5,and SOX-11 expression.Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement.Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved;these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease.In this case,positron emission tomography scanning and clinical staging revealed no systemic involvement,and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease.The prognosis of primary cutaneous MCL is relatively good.Our patient received six cycles of chemotherapy,and the cutaneous manifestations presented almost complete remission.CONCLUSION Primary cutaneous MCL is rare,and its prognosis is relatively favorable.However,correct diagnosis is a prerequisite for proper treatment.
基金the Beijing Key Clinical Subject Program and Beijing Municipal Administration of Hospitals Incubating Program,No.PX2018002.
文摘BACKGROUND QT interval prolongation can induce torsades de pointes(TdP),a potentially fatal ventricular arrhythmia.Recently,an increasing number of non-cardiac drugs have been found to cause QT prolongation and/or TdP onset.Moreover,recent findings have demonstrated the key roles of systemic inflammatory activation and fever in promoting long-QT syndrome(LQTS)and TdP development.CASE SUMMARY A 30-year-old woman was admitted with a moderate to high-grade episodic fever for two weeks.The patient was administered with multiple antibiotics after hospitalization but still had repeating fever and markedly elevated C-reactive protein.Once after a high fever,the patient suddenly lost consciousness,and electrocardiogram(ECG)showed transient TdP onset after frequent premature ventricular contraction.The patient recovered sinus rhythm and consciousness spontaneously,and post-TdP ECG revealed a prolonged QTc interval of 560 ms.The patient’s clinical manifestations and unresponsiveness to the antibiotics led to the final diagnosis of adult-onset Still’s disease(AOSD).There was no evidence of cardiac involvement.After the AOSD diagnosis,discontinuation of antibiotics and immediate initiation of intravenous dexamethasone administration resulted in the normal temperature and QTc interval.The genetic analysis identified that the patient and her father had heterozygous mutations in KCNH2(c.1370C>T)and AKAP9(c.7725A>C).During the 2-year follow-up period,the patient had no recurrence of any arrhythmia and maintained normal QTc interval.CONCLUSION This case study highlights the risk of systemic inflammatory activation and antibiotic-induced TdP/LQTS onset.Genetic analysis should be considered to identify individuals at high risk of developing TdP.