An immunohistochemical study of T lymphocyte subsets on frozen substituted plastic embedding bone marrow sections obtained from 10 patients with myelodysplastic syndrome (MDS) was presented. The results of qualitative...An immunohistochemical study of T lymphocyte subsets on frozen substituted plastic embedding bone marrow sections obtained from 10 patients with myelodysplastic syndrome (MDS) was presented. The results of qualitative and quantitative immunohistochemical analysis are as follows: (1) Labile antigens of T lymphocytes were well preserved, thus allowing analysis of distribution of T lymphocyte subsets in situ ; (2) the average number of T 3, T 4 and T 8 lymphocyte of the diffuse infiltrate was about 2 %, 0.4 %, 0.5 %, respectively, of all nucleated cells in bone marrow, and T 4/T 8 of T cells were below 1.0 in patients with MDS; (3) there were cases of RAS showing T lymphocyte aggregation in bone marrow, but no patient exhibited progressive refractory anemia with excess of blasts(RAEB) and RAEB in transformation (RAEBT). These findings indicated that the immunological abnormalities are of importance in the evaluation of pathogenesis and prognosis of MDS.展开更多
Acquired bone marrow failure diseases(ABMFD) are a class of hematopoietic stem cell diseases with a commonality of non-inherited disruption of hematopoiesis that results in pancytopenia. ABMFDs also are a group of het...Acquired bone marrow failure diseases(ABMFD) are a class of hematopoietic stem cell diseases with a commonality of non-inherited disruption of hematopoiesis that results in pancytopenia. ABMFDs also are a group of heterogeneous diseases with different etiologies and treatment options. The three most common ABMFDs are aplastic anemia, myelodysplastic syndrome, and paroxysmal nocturnal hemoglobinuria. Stem cell transplantation is the only treatment that can cure these diseases. However, due to high therapy-related mortality, stem cell transplantation has rarely been used as a first line treatment in treating ABMFD. With the advance of personalized medicine and precision medicine, various novel cellular therapy strategies are in trial to increase the efficiency and efficacy of ABMFD treatment. This article aims to review current available stem cell transplantation protocols and promising cellular therapy research in treating ABMFD.展开更多
Objective To determine the expression and function of the c-kit receptor in bone marrow mononuclear cells (BMMNC) of patients with myelodysplastic syndromes (MDS). Methods Direct immunofluorescence assay and reverse t...Objective To determine the expression and function of the c-kit receptor in bone marrow mononuclear cells (BMMNC) of patients with myelodysplastic syndromes (MDS). Methods Direct immunofluorescence assay and reverse transcriptase-polymerase chain reaction (RTPCR) were used to detect c-kit protein and c-kit mRNA expressions in the BMMNC of 29 MDS patients and 10 normal controls. Cell culture was used to detect the function of the c-kit receptor. Results c-kit protein expression in the MDS group was significantly higher than that in the control group (8.58％ +5.28％ vs 3.04％ + 1.49％, P＜0.05). c-kit protien expression in the refractory anemia (RA)group was significantly lower than that in the RA with an excess of blasts (RAEB)/RAEB in transformation (RAEB-t) group (5.12％ +2.13％ vs 10.01％ +5.07％, P＜0.05). The rate of c-kit protein expression was 32.43％ in aoute myeloblastic leukemia (AML) cases transformed from MDS (t-AML). c-kit mRNA expression in the MDS group was correlated with c-kit protein expression. Interieukin-3 (IL-3) and erythropoietin (Epo), with or without stem cell factor (SCF), upregulated c-kit protein and its mRNA expression. In the presence of IL-3 and Epo, SCF showed significant stimulating effects on the formation of CFU-GM and BFU-E in semi-solid cultures of normal BMMNC, but had no effects on those of the MDS patients.Conclusion The protein and mRNA expression of the c-kit receptor in the BMMNC of MDS patients were higher than those of normal controls, and the function of this receptor in MDS BMMNC was abnormal. Chin Med J 2001; 114(5) :481-485展开更多
BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoi...BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.展开更多
BACKGROUND Giant cellulitis-like Sweet syndrome(SS) is a rare subtype of SS,and reports of the combined histiocytoid type of pathology are scarce.Here,we report a case of SS with distinctive clinical presentations and...BACKGROUND Giant cellulitis-like Sweet syndrome(SS) is a rare subtype of SS,and reports of the combined histiocytoid type of pathology are scarce.Here,we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis.By sharing this case and a discussion of the related literature in detail,we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like(HGC)-SS and the pathogenesis of SS.CASE SUMMARY A 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swelling with pain for 1 d.He was diagnosed with myelodysplastic syndrome(MDS) and angioneurotic edema of the tongue and floor of the mouth.However,7 d after examination by sternal aspiration,a violaceous,tender,and swollen nodule developed at the site,with poorly demarcated erythema of the surrounding skin.Considering his profile of risk factors,the diagnosis of cellulitis was made and he was administered broad-spectrum antibiotics.When the lesion continued to worsen and he developed chills and fever,pathogenic and dermatopathological examination led to the diagnosis of HGC-SS.Treatment with prednisone led to the fever being relieved within 24 h and the skin lesion being resolved within 1 wk.The patient refused intensive treatment and was instead given thalidomide,erythropoietin,stanozolol,and supportive care.The prednisone was gradually tapered,with no signs of recurrence,but he died 2 mo later of severe pneumonia.CONCLUSION HGC-SS demonstrates unique manifestation.SS and leukemia cutis share cytological origin.Myelofibrosis and SS are adverse prognostic factors for MDS.展开更多
文摘An immunohistochemical study of T lymphocyte subsets on frozen substituted plastic embedding bone marrow sections obtained from 10 patients with myelodysplastic syndrome (MDS) was presented. The results of qualitative and quantitative immunohistochemical analysis are as follows: (1) Labile antigens of T lymphocytes were well preserved, thus allowing analysis of distribution of T lymphocyte subsets in situ ; (2) the average number of T 3, T 4 and T 8 lymphocyte of the diffuse infiltrate was about 2 %, 0.4 %, 0.5 %, respectively, of all nucleated cells in bone marrow, and T 4/T 8 of T cells were below 1.0 in patients with MDS; (3) there were cases of RAS showing T lymphocyte aggregation in bone marrow, but no patient exhibited progressive refractory anemia with excess of blasts(RAEB) and RAEB in transformation (RAEBT). These findings indicated that the immunological abnormalities are of importance in the evaluation of pathogenesis and prognosis of MDS.
基金Supported by AA and MDSIF research grant to Pu JJ,No.146818American Cancer Society grant to Pu JJ,No.124171-IRG-13-043-02a Pennsylvania State University College of Medicine research grant to Pu JJ
文摘Acquired bone marrow failure diseases(ABMFD) are a class of hematopoietic stem cell diseases with a commonality of non-inherited disruption of hematopoiesis that results in pancytopenia. ABMFDs also are a group of heterogeneous diseases with different etiologies and treatment options. The three most common ABMFDs are aplastic anemia, myelodysplastic syndrome, and paroxysmal nocturnal hemoglobinuria. Stem cell transplantation is the only treatment that can cure these diseases. However, due to high therapy-related mortality, stem cell transplantation has rarely been used as a first line treatment in treating ABMFD. With the advance of personalized medicine and precision medicine, various novel cellular therapy strategies are in trial to increase the efficiency and efficacy of ABMFD treatment. This article aims to review current available stem cell transplantation protocols and promising cellular therapy research in treating ABMFD.
文摘Objective To determine the expression and function of the c-kit receptor in bone marrow mononuclear cells (BMMNC) of patients with myelodysplastic syndromes (MDS). Methods Direct immunofluorescence assay and reverse transcriptase-polymerase chain reaction (RTPCR) were used to detect c-kit protein and c-kit mRNA expressions in the BMMNC of 29 MDS patients and 10 normal controls. Cell culture was used to detect the function of the c-kit receptor. Results c-kit protein expression in the MDS group was significantly higher than that in the control group (8.58％ +5.28％ vs 3.04％ + 1.49％, P＜0.05). c-kit protien expression in the refractory anemia (RA)group was significantly lower than that in the RA with an excess of blasts (RAEB)/RAEB in transformation (RAEB-t) group (5.12％ +2.13％ vs 10.01％ +5.07％, P＜0.05). The rate of c-kit protein expression was 32.43％ in aoute myeloblastic leukemia (AML) cases transformed from MDS (t-AML). c-kit mRNA expression in the MDS group was correlated with c-kit protein expression. Interieukin-3 (IL-3) and erythropoietin (Epo), with or without stem cell factor (SCF), upregulated c-kit protein and its mRNA expression. In the presence of IL-3 and Epo, SCF showed significant stimulating effects on the formation of CFU-GM and BFU-E in semi-solid cultures of normal BMMNC, but had no effects on those of the MDS patients.Conclusion The protein and mRNA expression of the c-kit receptor in the BMMNC of MDS patients were higher than those of normal controls, and the function of this receptor in MDS BMMNC was abnormal. Chin Med J 2001; 114(5) :481-485
基金the National Science of Foundation Committee of Zhejiang Province,No.LY22H160003the Zhejiang Provincial Medical and Health Science Foundation,No.2021441200 and No.2021RC083.
文摘BACKGROUND Hypereosinophilic syndrome(HES)is classified as primary,secondary or idiopathic.Idiopathic HES(IHES)has a variable clinical presentation and may involve multiple organs causing severe damage.Hepatic sinusoidal obstruction syndrome(HSOS)is characterized by damage to the endothelial cells of the hepatic sinusoids of the hepatic venules,with occlusion of the hepatic venules,and hepatocyte necrosis.We report a case of IHES with HSOS of uncertain etiology.CASE SUMMARY A 70-year-old male patient was admitted to our hospital with pruritus and a rash on the extremities for>5 mo.He had previously undergone antiallergic treatment and herbal therapy in the local hospital,but the symptoms recurred.Relevant examinations were completed after admission.Bone marrow aspiration biopsy showed a significantly higher percentage of eosinophils(23%)with approximately normal morphology.Ultrasound-guided hepatic aspiration biopsy indicated HSOS.Contrast-enhanced computed tomography(CT)of the upper abdomen showed hepatic venule congestion with hydrothorax and ascites.The patient was initially diagnosed with IHES and hepatic venule occlusion.Prednisone,low molecular weight heparin and ursodeoxycholic acid were given for treatment,followed by discontinuation of low molecular weight heparin due to ecchymosis.Routine blood tests,biochemical tests,and imaging such as enhanced CT of the upper abdomen and pelvis were reviewed regularly.CONCLUSION Hypereosinophilia may play a facilitating role in the occurrence and development of HSOS.
基金Supported by the National Natural Science Foundation of China,No. 82100125。
文摘BACKGROUND Giant cellulitis-like Sweet syndrome(SS) is a rare subtype of SS,and reports of the combined histiocytoid type of pathology are scarce.Here,we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis.By sharing this case and a discussion of the related literature in detail,we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like(HGC)-SS and the pathogenesis of SS.CASE SUMMARY A 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swelling with pain for 1 d.He was diagnosed with myelodysplastic syndrome(MDS) and angioneurotic edema of the tongue and floor of the mouth.However,7 d after examination by sternal aspiration,a violaceous,tender,and swollen nodule developed at the site,with poorly demarcated erythema of the surrounding skin.Considering his profile of risk factors,the diagnosis of cellulitis was made and he was administered broad-spectrum antibiotics.When the lesion continued to worsen and he developed chills and fever,pathogenic and dermatopathological examination led to the diagnosis of HGC-SS.Treatment with prednisone led to the fever being relieved within 24 h and the skin lesion being resolved within 1 wk.The patient refused intensive treatment and was instead given thalidomide,erythropoietin,stanozolol,and supportive care.The prednisone was gradually tapered,with no signs of recurrence,but he died 2 mo later of severe pneumonia.CONCLUSION HGC-SS demonstrates unique manifestation.SS and leukemia cutis share cytological origin.Myelofibrosis and SS are adverse prognostic factors for MDS.
