Hairy cell leukemia(HCL)is an uncommon mature B-cell malignancy characterized by a typical morphology,immunophenotype,and clinical profile.The vast majority of HCL patients harbor the canonical BRAF V600E mutation whi...Hairy cell leukemia(HCL)is an uncommon mature B-cell malignancy characterized by a typical morphology,immunophenotype,and clinical profile.The vast majority of HCL patients harbor the canonical BRAF V600E mutation which has become a rationalized target of the subsequently deregulated RAS-RAF-MEK-MAPK signaling pathway in HCL patients who have relapsed or who are refractory to front-line therapy.However,several HCL patients with a classical phenotype display non-canonical BRAF mutations or rearrangements.These include sequence variants within alternative exons and an oncogenic fusion with the IGH gene.Care must be taken in the molecular diagnostic work-up of patients with typical HCL but without the BRAF V600E to include investigation of these uncommon mechanisms.Identification,functional characterization,and reporting of further such patients is likely to provide insights into the pathogenesis of HCL and enable rational selection of targeted inhibitors in such patients if required.展开更多
The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions...The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions,which was treated successfully.We suggest that in the inves- tigation of discrete hepatic lesions in cases of cancer of unknown primary,hairy cell leukemia should be consid- ered.The excellent response of hairy cell leukemia to therapy highlights the need for such a consideration.展开更多
Hairy cell leukemia(HCL)is a rare B-cell lymphoproliferative disorder.Patients typically present with cytopenia and splenomegaly.We describe the case of a 78-year-old patient with refractory HCL who acutely developed ...Hairy cell leukemia(HCL)is a rare B-cell lymphoproliferative disorder.Patients typically present with cytopenia and splenomegaly.We describe the case of a 78-year-old patient with refractory HCL who acutely developed a cystic lesion on the back while receiving moxetumomab pasudotox therapy.Biopsy of the lesion revealed the presence of adenocarcinoma,which prompted a detailed evaluation resulting in a diagnosis of stage IV gastric cancer.Nevertheless,to establish any association between moxetumomab pasudotox therapy and secondary cancer development,a satisfactory number of studies need to be conducted.展开更多
Non-Hodgkin’s lymphoma cell leukemia (NHLCL),chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HLC) are the diseases very similar to each other. The differential diagnosis is very difficult,especially when ...Non-Hodgkin’s lymphoma cell leukemia (NHLCL),chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HLC) are the diseases very similar to each other. The differential diagnosis is very difficult,especially when there are small lymphoid cells in Periphcral blood and bone marrow under light microscope. We have observed 34 cases with electron microscope. The studies were correlated with clinical manifestation, cytology, pathology and immunologic histochemistry. Ultrastructural features strongly indicated the difference in three various diseases, although all the immunologic markers showed B-cell type.It is concluded that electron microscopic examination is of a definite significance in the diaguosis and successful treatment.展开更多
To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestat...To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestations, ultrastructure and immunophenotype were analyzed The results showed that hairy cell leukemia (HCL), splenic lymphoma with villous lymphocyte (SLVL) and hairy cell leukemia-variant (HCL-V) had some common characters including splenomegaly, peripheral blood and bone marrow infiltration by villous lymphocyte and B lymphocyte immunophenotype; but these three disorders had specific features respectively It was concluded that overall analysis of clinical and laboratory features might be contributive to the differential diagnosis of these three disorders展开更多
AIM： TO evaluate seven patients with non-traumatic splenic rupture （NSR）. NSR is an uncommon dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patient＇s sur...AIM： TO evaluate seven patients with non-traumatic splenic rupture （NSR）. NSR is an uncommon dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patient＇s survival. METHODS： Within 11 years, seven cases were evaluated for patient characteristics, anamnesis and symptoms, method of diagnosis, findings of laparotomy, and etiology of NSR. RESULTS： There were six （86%） male and one female （14%） patient, whose mean age was 36 ± 12.8 （17-56） years. We report here four cases of Plasmodium vivax malaria （cases Ⅰ-Ⅳ）, one case of hemodialysis （case Ⅴ）, one case of spontaneous splenic rupture （case Ⅵ）, and one case of hairy cell leukemia （case Ⅶ）. Splenectomy was performed in all patients. All of them made an uneventful recovery and were discharged in stable condition. CONCLUSION： NSR is a rare entity that needs a high index of suspicion for diagnosis. Using ultrasonography or computer tomography, and peritoneal aspiration of fresh blood may assist in the diagnosis of NSR. Increased awareness of NSR can enhance early diagnosis and effective treatment.展开更多
文摘Hairy cell leukemia(HCL)is an uncommon mature B-cell malignancy characterized by a typical morphology,immunophenotype,and clinical profile.The vast majority of HCL patients harbor the canonical BRAF V600E mutation which has become a rationalized target of the subsequently deregulated RAS-RAF-MEK-MAPK signaling pathway in HCL patients who have relapsed or who are refractory to front-line therapy.However,several HCL patients with a classical phenotype display non-canonical BRAF mutations or rearrangements.These include sequence variants within alternative exons and an oncogenic fusion with the IGH gene.Care must be taken in the molecular diagnostic work-up of patients with typical HCL but without the BRAF V600E to include investigation of these uncommon mechanisms.Identification,functional characterization,and reporting of further such patients is likely to provide insights into the pathogenesis of HCL and enable rational selection of targeted inhibitors in such patients if required.
文摘The involvement of hairy cell leukemia in the liver is in the form of portal and sinusoidal cellular infiltration. Here we describe the first case of hepatic hairy cell leukemia presenting as multiple discrete lesions,which was treated successfully.We suggest that in the inves- tigation of discrete hepatic lesions in cases of cancer of unknown primary,hairy cell leukemia should be consid- ered.The excellent response of hairy cell leukemia to therapy highlights the need for such a consideration.
文摘Hairy cell leukemia(HCL)is a rare B-cell lymphoproliferative disorder.Patients typically present with cytopenia and splenomegaly.We describe the case of a 78-year-old patient with refractory HCL who acutely developed a cystic lesion on the back while receiving moxetumomab pasudotox therapy.Biopsy of the lesion revealed the presence of adenocarcinoma,which prompted a detailed evaluation resulting in a diagnosis of stage IV gastric cancer.Nevertheless,to establish any association between moxetumomab pasudotox therapy and secondary cancer development,a satisfactory number of studies need to be conducted.
文摘Non-Hodgkin’s lymphoma cell leukemia (NHLCL),chronic lymphocytic leukemia (CLL) and hairy cell leukemia (HLC) are the diseases very similar to each other. The differential diagnosis is very difficult,especially when there are small lymphoid cells in Periphcral blood and bone marrow under light microscope. We have observed 34 cases with electron microscope. The studies were correlated with clinical manifestation, cytology, pathology and immunologic histochemistry. Ultrastructural features strongly indicated the difference in three various diseases, although all the immunologic markers showed B-cell type.It is concluded that electron microscopic examination is of a definite significance in the diaguosis and successful treatment.
文摘To identify the knowledge of rare lymphoproliferative disorder, the clinical and biological features of three kinds of lymphoproliferative disorders with cytoplasmic projections were compared The clinical manifestations, ultrastructure and immunophenotype were analyzed The results showed that hairy cell leukemia (HCL), splenic lymphoma with villous lymphocyte (SLVL) and hairy cell leukemia-variant (HCL-V) had some common characters including splenomegaly, peripheral blood and bone marrow infiltration by villous lymphocyte and B lymphocyte immunophenotype; but these three disorders had specific features respectively It was concluded that overall analysis of clinical and laboratory features might be contributive to the differential diagnosis of these three disorders
文摘AIM： TO evaluate seven patients with non-traumatic splenic rupture （NSR）. NSR is an uncommon dramatic abdominal emergency that requires immediate diagnosis and prompt surgical treatment to ensure the patient＇s survival. METHODS： Within 11 years, seven cases were evaluated for patient characteristics, anamnesis and symptoms, method of diagnosis, findings of laparotomy, and etiology of NSR. RESULTS： There were six （86%） male and one female （14%） patient, whose mean age was 36 ± 12.8 （17-56） years. We report here four cases of Plasmodium vivax malaria （cases Ⅰ-Ⅳ）, one case of hemodialysis （case Ⅴ）, one case of spontaneous splenic rupture （case Ⅵ）, and one case of hairy cell leukemia （case Ⅶ）. Splenectomy was performed in all patients. All of them made an uneventful recovery and were discharged in stable condition. CONCLUSION： NSR is a rare entity that needs a high index of suspicion for diagnosis. Using ultrasonography or computer tomography, and peritoneal aspiration of fresh blood may assist in the diagnosis of NSR. Increased awareness of NSR can enhance early diagnosis and effective treatment.
