Background The classical theory from Gass holds that idiopathic macular hole (IMH) is due to the dragging of vitreous cortex in the tangent direction on the macular region,while spectral domain OCT (SD-OCT)studies found that the diameters of inner segment/outer segment (IS/OS) absence (DIOA) region was closely associated with vision ability of IMH patients.Enhanced depth imaging (EDI) is thought to be a new method to study the relation between DIOA and subfoveal choroidal thickness (SFCT) ,but there are few relevant reports.Objective This study aimed to observe the correlation between photoreceptor IS/OS junction and SFCT before and after vitrectomy in IMH by EDI SD-OCT.Methods Forty unilateral IMH patients were enrolled in Affiliated Foshan Hospital of Southern Medical University from June 2011 to June 2013.Vitrectomy (23G) was performed on the patients by the same operator.EDI mode of SD-OCT was used to measure DIOA and SFCT in horizontal direction before and 6 months after surgery.The relationship between DIOA and SFCT was evaluated using Pearson linear correlation analysis.Written informed consent was obtained from each patient before medical assessment.Results IMH was anatomically closed after vitrectomy in 34 eyes with the closure rate 85％.The mean DIOA was (1 280±753) μm in preoperation, which was significantly higher than (656 ± 322) μm in postoperation (t =4.989, P =0.000).The mean SFCT was (130±43)μm in preoperation,showing a slight reduce in comparison with (140±38)μrn in postoperation, but there was not statistical significance between them (t =-1.407, P =0.175).Negative correlations were found between DIOA and SFCT in both preoperation and postoperation (preoperation:r=-0.748 ,P=0.000;postoperation: r=-0.686,P =0.001).Conclusions The alteration of DIOA shows a negative correlation with SFCT in IMH eyes,suggesting a contributing role of the choroid in the change of photoreceptors of IMH.

Objective To improve the acknowledge of diagnosis and therapy of aggressive systemic mastocytosis (ASM).Methods One ASM patient was reported and the literatures were reviewed.Results As a rare subtype of SM,ASM is characterized by multiple organs involvement,and often accompanied by bone marrow dysfunction,osteolytic lesions and palpable hepatomegaly or splenomegaly which usually indicate the high mast cell burden.Conclusion ASM meets criteria for SM and has one or more C findings.Variable factors affect the prognosis of ASM patients and the formulation of the clinical treatment strategy which leads to the highly individualized therapies.

Objective To investigate the changes and prognostic significance of bone marrow(BM) oil drop and megakaryocyte counts after chemotherapy in acute myeloid leukemia (AML) patients (non-M3).Methods Ninty-nine adult patients with denovo AML (non-M3) were retrospectively analyzed to evaluate the change of BM oil drop and megakaryocyte counts and their influences on overall survival(OS) and disease free survival (DFS) during all stages of standardized therapy.Results The median DFS and OS were 21 (2-88);months and 70 (4-89) months,respectively; and 3-year predicted DFS and OS were 47.3 ％ and 55.8 ％,respectively.After AML patients (non-M3) achieving complete remission (CR) by induction therapy,BM oil drop tended to increase along with postremission chemotherapy cycle accumulation, while megakaryocyte counts tended to decrease.The univariate analysis indicated that megakaryocyte counts decreased after the second course of postremission therapy. BM oil drop increased after the first to the third course of postremission therapy.Grade of myelofibrosis in BM biopsy,serum lactate dehydrogenase (LDH) level at diagnosis,flow cytometric immunophenotyping, the percentage of BM blast cells at diagnosis and the percentage of residual leukemic cells (RLC) during aplasia (7-10 days after the end of induction therapy) had prognostic significance.Multivariable COX analysis indicated the percentage of BM blast cells at diagnosis and change of BM oil drop after the third postremission therapy were independent prognostic factors for DFS (P =0.010,0.018 respectively),and RLCs during aplasia and change rate of the megakaryocyte counts after the second postremission therapy were independent prognostic factors for OS (P =0.009, 0.038respectively).Conclusion After AML patients (non-M3) achieving CR by induction therapy,BM oil drop tends to increase along with postremission chemotherapy cycles accumulation,while the megakaryocyte counts tend to decrease.Dynamic observations of bone marrow oil drop and megakaryocyte counts are helpful for assessing the prognosis of acute myeloid leukemia (non-M3).

Objective:To investigate the clinical features, the key point of diagnosis and treatment methods of X-linked hyper-IgM syndrome (XHIGM).Methods:The clinical characteristics and laboratory data of a patient aged 23 years who was diagnosed as XHIGM complicated with T-cell large granular lymphocytic leukemia (TLGLL) in Institute of Hematology & Blood Diseases Hospital in March 2020 were analyzed retrospectively, and the literatures were reviewed.Results:This male patient presented with recurrent infection when he was 17 years old, and was found neutropenia, anemia accompanied by obvious splenomegaly, lower level of IgG and IgA after the visit. The level of IgM was lower than the normal level and the typical XHIGM was manifested with the normal or increased level of IgM, however CD40L homozygous mutation (chromosome: chrX; location: 135730438; variation of amino acid: NM_000074:exon1:c.31C>T:p.R11X; nonsense mutation) was confirmed by next generation sequencing. CD40L heterozygous mutation was detected in his mother, but it was not in his father. The patient was diagnosed as XHIGM. Anemia and neutropenia were alleviated after splenectomy in the patient, who was diagnosed as T-cell large granular lymphocyte elevation and clonal proliferation by flow cytometry, TCR gene rearrangement positive and bone marrow histopathological immunohistochemistry results because of the increasing leukocyte. The patient was eventually diagnosed as XHIGM complicated with T-LGLL.Conclusions:A small number of patients with XHIGM may develop symptoms in adulthood and may present with atypical clinical features of significant reduction in IgG, IgA, and IgM. The confirmed diagnosis of XHIGM is established by identification of CD40L gene mutation. XHIGM gene screening is required in male patients with recurrent infection, IgG level lower than normal and neutropenia. A few XHIGM patients are complicated with T-LGLL.

Objective To investigate the features of computed tomography (CT) and magnetic resonance imaging (MRI) of regional portal hypertension (RPH).Methods The retrospective cohort study was conducted.The clinicopathological data of 31 patients with PHR in the RPH group and 31 patients with liver cirrhotic portal hypertension (CPH) in the CPH group who were admitted to the Affiliated Hospital of Inner Mongolia Medical University between February 2014 and February 2018 were collected.Etiologies of patients in the RPH group included 21 of chronic pancreatitis complicated with pancreatic pseudocyst,5 of carcinoma of pancreatic body and tail,1 of solid pseudopapillary tumor of the pancreas,1 of pancreatic serotls cystoadenoma,1 of gastric stromal tumor,1 of retroperitoneal metastatic tumor and 1 of left renal carcinoma.Etiologies of patients in the CPH group included 27 of liver cirrhosis after viral hepatitis type B (4 complicated with liver metastasis),3 of alcoholic cirrhosis and 1 of cholestatic cirrhosis.All the patients underwent CT and MRI examinations.Patients in the RPH group were mainly treated the primary diseases and patients in the CPH group were decreased portal vein pressure.Observation indicators:(1) imaging features of patients in the two groups;(2) treatment and follow-up situations.Follow-up using outpatient examination and telephone interview were performed to detect management of portal hypertension after treatment up to February 2018.Measurement data with normal distribution were represented as (x)±s and comparison between groups was analyzed by the t test.Measurement data were represented as M (range) and comparison between groups was analyzed by the Mann-Whitney rank sum test.Count data were compared with chi-square test.Results (1) Imaging features of patients in the two groups:of 31 patients in the RPH group,12 underwent CT examination,2 underwent MRI examination,and 17 underwent CT combine with MRI examination.Of 31 patients in the CPH group,12 underwent CT examination and 19 underwent CT combined with MRI examination.The number of patients with varices in the gastric fundus,the number of patients with combined esophageal varices,the number of perigastric varices,diameter of main portal vein,diameter of splenic vein,liver volume,splenic volume,hepatosplenic volume ratio were 11,1,49,(13.9±2.9) mm,(12.0±2.8) mm,1 383 cm3 (range,1 005-1 637 cm3),271 cm3(range,199-311 cm3) and 5.5±2.0 in the RHP group and 24,21,33,(16.3±1.7)mm,(10.5±3.2)mm,1 087 cm3(range,916-1 536 cm3),603 cm3(range,415-869 cm3) and 2.2±0.9 in the CHP group,with statistically significant differences in the number of patients with varices in the gastric fundus,the number of patients with combined esophageal varices,the number of perigastric varices,diameter of main portal vein,splenic volume,hepatosplenic volume ratio between the two groups (x2=11.088,28.182,8.940,t=4.430,Z=6.205,t=8.544,P＜0.05) and with no statistically significant differences in the diameter of splenic vein and liver volume between the two groups (t=1.974,Z=1.162,P＜0.05).Of 31 patients in the RPH group,2 with pancreatic pseudocyst were misdiagnosed as pancreatic cancer and 29 were diagnosed accurately by imaging examinations.Of 31 patients in the CPH group,3 with liver metastasis were undetected by CT examination and the other 28 were diagnosed accurately by imaging examinations.Splenic vein occlusion,severe splenic vein stenosis,moderate splenic vein stenosis and mild splenic vein stenosis were detected in 2,17,10 and 2 of 31 patients in the RHP group.All the 31 patients in the CHP group mainly had dilation in splenic veins,with no clear stenosis.(2) Treatment and follow-up situations:patients in the two groups were followed up for 6-48 months,with a median time of 21 months.Of 21 patients with chronic pancreatitis complicated with pancreatic pseudocyst in the RPH group,7 underwent pancreatic pseudocyst puncture and drainage,6 of them had poor control on portal hypertension and 1 had moderate control;4 underwent pancreaticoenteric drainage,1 of them underwent pancreaticojejunostomy 4 years later and 3 of them had good control on portal hypertension;3 undergoing splenectomy combined with perigastrectomy had good control on portal hypertension;7 undergoing conservative treatment had good control on portal hypertension.Of 5 patients with carcinoma of pancreatic body and tail in the RHP group,2 undergoing distal pancreatectomy combined with splenectomy had good control on portal hypertension and 3 undergoing non-operative combined therapy died of primary disease one year later.One,1 and 1 patient with solid pseudopapillary tumor of the pancreas,pancreatic serotls cystoadenoma and gastric stromal tumor respectively in the RHP group underwent relative surgical treatments and had good control on portal hypertension.One and 1 patient with retroperitoneal metastatic tumor and left renal carcinoma respectively in the RHP group underwent non-operative combined therapy and had good control on portal hypertension.All the 31 patients in the CHP group were mainly treated with protection of liver function,8 of them were encounted with medusa head,7 with upper gastrointestinal rehemorrhage within one year,5 with subcutaneous varicose vein of abdominal wall,3 with continuing increase of spleen volume and 8 had good control on portal hypertension.Conclusions RHP are existed in pancreatic,splenic or peritoneal diseases,especially the pancreatic primary diseases.The main imaging features of RHP include isolated gastric varices,perigastric varices and splenic vein occlusion without normal main portal vein and liver function.Surgical resection of primary tumor and reasonable splenectomy are effective therapy.

Objective:To summarize the computed tomography (CT) and magnetic resonance imaging (MRI) features of acinar cell carcinoma of the pancreas (ACCP).Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 21 patients with ACCP who were admitted to the Affiliated Hospital of Inner Mongolia Medical University from January 2015 to December 2019 were collected. There were 5 males and 16 females, aged (57±9)years, with a range from 41 to 74 years. Patients underwent CT and MRI examinations. Observation indicators: (1) imaging examination; (2) imaging features on CT; (3) imaging features on MRI; (4) pathological examination and immunohistochemistry staining; (5) treatment and follow-up. Follow-up using outpatient examination and telephone interview was conducted at 1, 3, 6 months after discharge and once every 6 months thereafter to detect survival of patients up to December 2019. Measurement data with normal distribution were represented as Mean± SD. Count data were described as absolute numbers. Results:(1) Imaging examination. Of the 21 patients, 7 underwent single CT examination, 11 underwent MRI examination, and 3 underwent both CT and MRI examinations. ① Tumor shape: all the 21 patients had single tumor, including 17 showing round or quasi-round shape, and 4 showing irregular clumps. ② Tumor location: of the 21 patients, 6 had tumor located at pancreatic head, 2 had tumor located at pancreatic head and body, 2 had tumor located at pancreatic body, 4 had tumor located at pancreatic body and tail, 4 had tumor located at pancreatic tail, and 3 had had tumor located at ampulla. ③ The maximum tumor diameter was (43±29)mm, with a range from 11 to 129 mm. ④ Adjacent organ invasion: 10 of the 21 patients had invasion of adjacent organ, including 2 with invasion of stomach, spleen and left adrenal gland invasion, 4 with invasion of duodenum, 3 with invasion of duodenum and common bile duct, 1 with invasion of spleen. ⑤ Vascular invasion: 12 patients had invasion of splenic artery or splenic vein, including 1 combined with invasion of both common hepatic artery and superior mesenteric vein, 1 combined with invasion of celiac root. ⑥ Pancreatic and bile duct invasion: 8 patients had pancreatic and bile duct dilation, including 4 with bile duct and upper pancreatic duct dilation, and 4 with pancreatic duct dilation. ⑦ Lymph node metastasis: 2 patients had perineoplastic lymph node enlargement. ⑧ Other conditions: 7 patients had tumor center with cystic necrosis. Four patients had atrophy pancreatic parenchyma. Two patients had splenic vein tumor thrombosis. Two patients had cysts. One patient had multiple liver metastases. (2) Imaging features on CT. ① The solid part was dominant in the main body of the 10 patients undergoing CT examination, demostrating equal density, of which 3 cases had clear boundaries, 2 cases had pseudocapsule around the lesion, and 5 cases had low-density necrotic area in the center of lesion. ② In arterial phase of CT examination, the solid part of tumor had a lower enhancement compared with the normal pancreatic tissues in 7 patients, while the solid part of tumor had a high enhancement compared with the normal pancreatic tissues in 3 patients. ③ In delayed phase of CT examination, the tumor density was slightly lower than or equal to density of normal pancreatic parenchyma in 7 patients, showing slightly progressive enhancement, while the tumor density was slightly higher than or equal to density of normal pancreatic parenchyma in 3 patients. (3) Imaging features on MRI. ① MRI plain scan of 14 patients showed that 8 patients demostrated slightly longer T2 and slightly longer T1 signals in lesions, while 6 patients demostrated mixed signals dominated by long T2 and equal T1 signals. The area of cystic necrosis was observed in lesions of 4 patients and was not observed in 10 patients. No antiphase signal reduction was observed in the 14 patients. ② MRI dynamic enhanced scan of 12 patients showed that 11 patients presented mild progressive enhancement in lesions and 1 patient presented obvious confounding enhancement and clearance in the delayed phase. Compared with adjacent normal pancreatic parenchyma, diffused weighted imaging showed high signals in 6 cases, slightly high signals in 6 cases, and high signal halo in 2 cases. The apparent diffusion coefficient in 14 lesions was (1.22±0.14)×10 -3 mm 2/s. (4) Pathological examination and immunohistochemistry staining. Results of pathological examination in the 21 patients: acinic cell carcinoma, mixed ductal-acinic cell carcinoma, acinar-endocrine carcinoma, and atypical hyperplasia inacinus were detected in 14, 5, 1, and 1 patients, respectively. Of the 21 patients, 10 had invasion of adjacent organ, 3 had invasion of bile duct, 2 had invasion of lymph node. Results of immunohistochemistry staining in 17 patients: 17 patients had proliferation index of Ki-67 as 1%-80%; 10 out of 16 patients were positive for synaptophysin; 6 out of 16 patients were positive for CD56 protein; 2 out of 14 patients were positive for Chromogranin A; 12 out of 13 patients were positive for α-antitrypsin; 9 out of 11 patients were positive for cytokeratin; 8 patients were positive for β-catenin; 2 patients were positive for B lymphoma-10 protein. (5) Treatment and follow-up. Of the 21 patients, 10 cases underwent pancreatico-duodenectomy, 6 cases underwent pancreatic body and tail pancreatectomy combined with splenectomy, 2 cases underwent pancreatic body and tail pancreatectomy, 1 case underwent pancreatic tail tumor enucleation, 1 case underwent liver metastasis resection, and 1 case underwent ultrasound-guided pancreatic lesion puncture biopsy. All the 21 patients were followed up for (30±16)years, with a range from 2 to 52 months. There were 13 patient surviving and 8 cases of death. They had survived for (19±13)months, with a range from 2 to 35 months. Conclusions:The CT and MRI enhanced scan of ACCP showed slightly progressive enhancement, with cystic necrosis seen in the center and high signals in diffused weighted imaging. Dilation of bile duct and pancreatic duct is common in patients with pancreatic head tumors, and invasion of splenic artery and vein is common in pancreatic body and tail tumors. Calcification and cyst are rare and lesions of pancreatic head and body cause atrophy in pancreatic tail.

Objective: To observe the clinical characteristics, treatment responses and prognosis of patients with myelodysplastic syndrome (MDS) -del (5q) syndrome who met WHO (2016) diagnostic typing criteria. Methods: A total of 77 patients with del (5q) syndrome, according to WHO (2016) classification, were retrospectively analyzed between January 2008 and April 2018 in the Blood Diseases Hospital, Chinese Academy of Medical Sciences. Clinical characteristics, lenalidomide (LEN) efficacy and survivals were compared between the patients with del (5q) alone and those with one additional cytogenetic abnormality (ACA) with the exception of monosomy 7 or del (7q) . Treatment response and overall survival (OS) were compared between patients who were treated with LEN and traditional non-LEN drugs. Results: Of 77 patients, 64 were isolated del (5q) and 13 were del (5q) with ACA. There were significant differences of the median age and percentage of patients who had small megakaryocytes in bone marrow smear by immunohistochemistry (CD41) between the patients with isolated del (5q) and the patients with del (5q) + ACA[58 (29-64) years old vs 63 (31-82) years old, z=2.164, P=0.030; and 91.7%vs 60.0%, P=0.046, respectively]. The overall hematological response rate (78.9%vs 80.0%) , complete hematological remission (CR) rate (57.9% vs 60.0%) , cytogenetic response (CyR) rate[69.2% (9/13) vs 66.7% (4/6) ] and complete cytogenetic response (CCyR) rate [61.5% (8/13) vs 33.3% (2/6) ] of LEN were similar between the patients with isolated del (5q) (n=19) and with del (5q) + ACA (n=10) , as well as the median Overall survival (OS) between these two groups of patients (62 months vs 78 months, P=0.388) . The hematological response rate (79.3% vs 36.0%) , CR rate (58.6% vs 8.0%) , CyR rate [68.4% (13/19) vs 11.1% (1/9) ] and CCyR rate [52.6% (10/19) vs 0 (0/9) ] were higher among patients treated with LEN (n=29) than those treated with non-LEN therapy (n=25) . There was no statistically significant difference in OS between the patients with LEN or non-LEN therapy (78 months vs 62 months, P=0.297) . Conclusion Comparing del (5q) syndrome patients with isolated del (5q) or with del (5q) + ACA, two groups of patients had similar clinical characteristics, median OS and LEN efficacy. LEN showed better treatment response than traditional drugs in patients with del (5q) syndrome.

Objective: To evaluate the efficacy and tolerability of ruxolitinib combined with prednisone, thalidomide and danazol for treatment of in myelofibrosis (MF). Methods: Patients of MF according to the WHO 2016 criteria, received ruxolitinib (RUX) combined with prednisone, thalidomide and danazol (PTD). The response, changes of blood counts and adverse events were evaluated. Results: Six PMF and one post-ET MF patients were enrolled. Four patients presented JAK2V617F mutation, one CALR mutation, one MPL mutation, one triple-negative. Responses per IWG-MRT criteria were clinical improvement in 5 patients, stable disease in 2 ones, spleen response in 6 ones. All of 7 patients were symptomatic responses, four patients achieved at least 50% improvement from baseline on MPN-SAF TSS. Three patients initially treated with RUX alone, all of 3 patients experienced treatment-associated anemia and thrombocytopenia. Then these 3 patients received RUX combined with PTD, both hemoglobin and platelet increased significantly. Four patients initially treated with RUX combined with PTD. Increased levels of hemoglobin and platelet were seen in all of 7 patients received RUX combined with PTD with maximum increased hemoglobin of 30(18-54) g/L and maximum increased platelets of 116(13-369)×10⁹/L, respectively from baseline. The treatment dose of RUX increased due to improved platelet count in 3 patients. The frequent non-hematologic adverse events grade 1-2 were constipation, abdominal distension, crura edema and increased ALT. Conclusions RUX combined with PTD for treatment of MF may modulate initial hematologic toxicity observed when RUX alone, and may increase response due to improved levels of hemoglobin or platelet.

AIM: To compare the differences in the efficacy and safety of combination of intravitreal dexamethasone(Ozurdex)and ranibizumab or monotherapy of ranibizumab in eyes with macular edema secondary to retinal vein occlusion(RVO-ME).METHODS: Patients diagnosed with non-ischemic RVO-ME by fluorescein fundus angiography in our hospital from June 2020 to December 2022 were selected. All patients were initially treated with intravitreal injection of ranibizumab(0.5 mg), and 42 patients(42 eyes)who had central retinal thickness(CRT)≥300 μm after 2 wk were included. They were randomly divided into combined treatment group and monotherapy group. The combined treatment group(21 eyes)received Ozurdex intravitreal injection immediately, while the monotherapy group(21 eyes)was treated with ranibizumab intravitreal injection by 3+pro re nata(PRN). The changes of best corrected visual acuity(BCVA), CRT, and intraocular pressure before and at 2 wk, 1, 2, 3, 4, 5, and 6 mo after treatment were recorded, and the ocular or systemic complications were observed.RESULTS:The BCVA and CRT of all patients at 2 wk, 1, 2, 3, 4, 5, and 6 mo after treatment were significantly better than those before treatment(all P<0.01). There were statistical significance in the BCVA and CRT between two groups at 2 and 3 mo after treatment(all P<0.05). The most significant increase of BCVA in the combined treatment group occurred at 2 mo after treatment. The mean recurrence time of macular edema in the monotherapy group was 1.45±0.53 mo, with 4.21±0.78 injection times of ranibizumab. None of the patients showed serious complications after treatment. The most common complications in the combined treatment group were subconjunctival hemorrhage and elevated intraocular pressure, which were manageable with topical ocular hypotensive agents, and no patient required antiglaucoma or cataract surgery.CONCLUSION: Compared with monotherapy of ranibizumab, intravitreal injection of dexamethasone combined with ranibizumab can significantly improve the visual acuity and effectively reduce the macular edema in the treatment of RVO-ME, with a long duration of efficacy and less intravitreal injection of drugs.

Objective: To observe the clinical efficacy and safety of the patients of myelodysplastic syndromes-refractory anemia with excess blasts (MDS-REAB) treated with decitabine alone or based on low dose cytarabine (Ara-C) regimen CAG/HAG [aclarubrci (ACR) /homoharring-tonine (HHT) +cytarabine+granulocyte colony stimulating factor (G-CSF) ]. Methods: Totally 121 patients with MDS-REAB were retrospectively analyzed, including 59 patients treated with decitabine alone (20 mg·m^-2·d^-1 for 5 days) , the rest 62 ones treated with low-dose Ara-C-based regimen CAG/HAG. Overall response rate (ORR) , overall survival (OS) and adverse events of the two groups were analyzed and compared retrospectively. Results: The ORR of decitabine alone or CAG/HAG were 66.2% and 56.4% respectively, with no statistically significant differences (χ²=1.185, P=0.276) . Initial response rate detected by the end of first cycle of CAG/HAG was higher than that of decitabine alone (94.3% vs 69.2%) , there was statistically significant difference in the overall comparison of two groups (χ²=7.612, P=0.009) . The median OS of decitabine alone was 19.5 (95% CI 10.5-28.4) months, the median OS of CAG/HAG was 20.3 (95% CI 10.7-29.9) months, with no statistically significant differences (χ²=0.004, P=0.947) . Grade 3-4 cytopenia and infection were the most prevalent adverses of two group patients. Grade 3-4 cytopenia rate of CAG/HAG was higher than that of decitabine alone (100.0% vs 64.4%, P<0.001) . The infection rate detected at third cycle of CAG/HAG was higher than that of decitabine alone (52.9% vs 15.2%, P=0.008) . Conclusion The efficacy of treating MDS-RAEB with decitabine alone or CAG/HAG was equivalent. CAG/HAG treatment came into effect faster, but decitabine alone treatment was safer.