Objective To explore the features of patients with acute leukemia (AL) in Hematology Department of Shenzhen Hospital affliated to Beijing University during the past six years. Methods The data of the 1056 acute leukemia dignosed in our department between 2002-2007 were collected and analyzed retrospectively. Results The male patients was predominant (male/female ratio was 1.78). The number of patients with acute myeloid leukemia(AML) was higher than that of patients with acute lymphoblastic leukemia (ALL) (AML/ALL ratio was 1.85). Most of the AML patients were young adult (65 %) and elderly ALL patients were very rare(1.33 %). AL cases were tended to increase gradually by year. The ratio of treatment-related AL in Shenzhen was lower than that reported literature in other countries. The major cause of the treatment-related AL were chemotherapy/radiotherapy and psoriasis treated with bimolane. Conclusion Some of the features of AL patients in China were further confirmed and some new features were also found in this study. These changes should be demonstrated by the next national epidemiology survey of leukemia.

To explore the clinical characteristics, diagnosis, treatment outcome and prognosis of de novo CD5 positive diffuse large B cell lymphoma (CD5(+)DLBCL), clinical data of 10 patients with pathologically confirmed CD5(+)DLBCL were retrospectively analyzed. The results indicated that 9 out of 10 patients were older than 60 years. All cases were in III/IV stages according to Ann-Arbor Staging System. Bone marrow biopsy with immunohistochemistry showed lymphoma involvement in 5 cases. Nine patients received chemotherapy with anti-CD20 monoclonal antibody (Rituximab) except one. Five cases achieved CR, two cases achieved PR, two cases achieved SD, one case achieved PD. Eight cases died within 2 years because of relapse or disease progression, in which 3 cases developed central nervous system lymphoma. The median survival time was 16 (1-23) months, 2-year survival rate was 20.40%. It is concluded that de novo CD5(+) DLBCL is rare in clinic, but it is a kind of highly aggressive lymphoma with poor prognosis. So, new treatment strategy should be explored.
Aged ; Aged, 80 and over ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; CD5 Antigens ; metabolism ; Female ; Humans ; Lymphoma, Large B-Cell, Diffuse ; drug therapy ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Staging ; Prognosis ; Retrospective Studies ; Rituximab ; Survival Rate ; Treatment Outcome

BACKGROUNDHypertensive crisis could be found after operation in patients with hypertensive intracerebral hemorrhage (HICH). The aim of this study was to explore the changes and the roles of some vasoactive polypeptides during postoperative hypertensive crisis in patients with HICH.

METHODSA total of 31 patients, who were admitted for craniotomy, were enrolled into this study. After the operation, the patients were divided into three groups. Group I consisted of 9 patients with postoperative hypertensive crisis, and group II was composed of 13 patients without postoperative hypertensive crisis. Nine patients, who denied history of hypertension or HICH, were set as group III. The levels of some vasoactivators in the three groups were measured before and after the operation. The differences in the results among the groups were analyzed using the ANOVA. The data collected before and after the operation in the group I was compared by Wilcoxon test.

RESULTSThe concentration of endothelin in group I was significantly higher than that in group III (P < 0.05). The level of thromboxane A2 and the ratio of thromboxane B2 to 6-keto-PGF1a in group I were significantly higher than those in the other two groups (P < 0.05). In group I, the levels of plasma renin activity, angiotensin II, aldosterone, catecholamine, and endothelin before the operation were significantly higher than those determined after the operation (P > 0.05).

CONCLUSIONSPostoperative hypertensive crisis may be due to the increased thromboxane A2 and relatively inadequate prostacyclin, especially 6-keto-PGF1a. The increased level of endothelin and intraoperative stimulation also play a certain role in the development of postoperative hypertensive crisis.

6-Ketoprostaglandin F1 alpha ; blood ; Adult ; Aged ; Endothelins ; blood ; Female ; Humans ; Hypertension ; blood ; etiology ; Intracranial Hemorrhage, Hypertensive ; blood ; physiopathology ; surgery ; Male ; Middle Aged ; Postoperative Complications ; blood ; Thromboxane B2 ; blood

The aim of this study was to analyze the clinical features and laboratory findings of adult Epstein-Barr virus associated T/NK cell lymphoproliferative disease (EBV+T/NK-LPD) and to investigate the early diagnosis and prognosis of EBV+T/NK-LPD. The clinical data of 19 adult patients with EBV+T/NK-LPD were retrospectively analyzed. The results indicated that there were 11 males and 8 females. The median age was 32 years (range: 20-70 years). The average duration from onset of symptoms to diagnosis was 3.5 months. The median survival time was 2.5 months. Unkown fever, hepatosplenomegaly, liver dysfunction and interstitial pneumonia were the main clinical features. High levels of β2-MG, LDH, TNF, IL-6 and significantly increased EBV-DNA level (median level > 10(6) copies/ml) were occurred in all the patients. Cytopenia was seen in 18 cases. Morphologically, atypical large granular lymphocytes and hemophagocytosis were common in bone marrow smears. Deletion of CD5 or CD7 were frequently observed in T/NK lymphocytes in bone marrow cells by flow cytometry. Bone marrow biopsy showed atypical lymphocyte interstitial infiltrated in 10 cases, while a few large cells infiltrated in 6 cases. Immunohistochemistry showed the expression of CD3(+)CD56(+) were seen in 2 cases, CD3(+)CD8(+) in 11 cases and CD3(+)CD4(+) in 3 cases. TIA-1 and EBER were positive in all biopsy specimens. Three cases underwent biopsy of lymph nodes showed reactive proliferations of lymphocytes. All the patients died of multiorgan failure. It is concluded that the fever, hepatosplenomegaly are the most common clinical features in adult EBV+T/NK-LPD, the bone marrow infiltration of EBV-infected T/NK lymphocytes and significantly increased EBV-DNA level can be observed in all cases, the clinical outcome of this disease is poor, these clinical and experimental features can be served as a reliable marker for the timely diagnosis of adult EBV+T/NK-LPD.
Adult ; Aged ; Epstein-Barr Virus Infections ; pathology ; Female ; Humans ; Immunophenotyping ; Killer Cells, Natural ; virology ; Lymphoproliferative Disorders ; pathology ; virology ; Male ; Middle Aged ; Retrospective Studies ; T-Lymphocytes ; virology ; Young Adult

Objective: To investigate the clinicopathologic characteristics of intractable epilepsy. Methods: Based on the classification criteria proposed by the International League Against Epilepsy (ILAE), a retrospective analysis of the pathological characteristics was done in 822 patients who underwent epilepsy surgery in Sanbo Brain Hospital, Capital Medical University, from June 2008 to December 2012. Results: The mean age of epilepsy onset was 9.9 years, mean duration of epilepsy was 11.9 years. Complex partial seizures were the main presenting features. Histopathological study showed 33 cases (4.01%) with mild forms of cortical malformations, 690 cases (83.94%) with focal cortical dysplasia (FCD) and 99 cases with others (including 39 pure hippocampal sclerosis, 20 cystosclerosis, 19 Sturge-Weber syndrome, 8 tuberous sclerosis complex, 6 without significant pathological changes, 5 gyral malformations and 2 hamartoma). Among the 690 FCD cases, 106 were FCD typeⅠ, 91 were FCD typeⅡ and 493 were FCDⅢ(Ⅲa: 160, Ⅲb: 106, Ⅲc: 26 and Ⅲd: 201). Conclusions FCDⅢd is the most common histopathological subtype causing intractable epilepsy, mainly due to focal hypoxia/ischemia in the perinatal period, which results in scarring of local brain tissue; this is followed by other isolated forms of FCD (FCDⅠand FCDⅡ), and then FCD Ⅲa and FCD Ⅲb. The reason to distinguish isolated forms of FCD (types Ⅰ and Ⅱ) from FCD Ⅲ and to subclassify FCD Ⅲ is to allow better definition of cortical dyslamination. Therefore, the pathogenic factors of intractable epilepsy can be grouped in greater details, and facilitate the diagnosis and potential curative treatment of intractable epilepsy.

BACKGROUNDPDK1 is an essential protein kinase that plays a critical role in mammalian development. Mouse lacking PDK1 leads to multiple abnormalities and embryonic lethality at E9.5. To elucidate the role of PDK1 in the heart, we investigated the cardiac phenotype of mice that lack PDK1 in the heart in different growth periods and the alteration of PDK1 signaling in human failing heart.

METHODSWe employed Cre/loxP system to generate PDK1(flox/flox): α-MHC-Cre mice, which specifically deleted PDK1 in cardiac muscle at birth, and tamoxifen-inducible heart-specific PDK1 knockout mice (PDK1(flox/flox):MerCreMer mice), in which PDK1 was deleted in myocardium in response to the treatment with tamoxifen. Transmural myocardial tissues from human failing hearts and normal hearts were sampled from the left ventricular apex to analyze the activity of PDK1/Akt signaling pathways by Western blotting.

RESULTSPDK1(flox/flox): α-MHC-Cre mice died of heart failure at 5 and 10 weeks old. PDK1(flox/flox) -MerCreMer mice died of heart failure from 5 to 21 weeks after the initiation of tamoxifen treatment at 8 weeks old. We found that expression levels of PDK1 in human failing heart tissues were significantly decreased compared with control hearts.

CONCLUSIONOur results suggest that PDK1 signaling network takes part in regulating cardiac viability and function in mice, and may be also involved in human heart failure disease.

3-Phosphoinositide-Dependent Protein Kinases ; Adult ; Animals ; Female ; Glycogen Synthase Kinase 3 ; physiology ; Heart ; physiology ; Heart Failure ; enzymology ; etiology ; Humans ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Middle Aged ; Myosin Heavy Chains ; physiology ; Protein-Serine-Threonine Kinases ; metabolism ; Proto-Oncogene Proteins c-akt ; physiology ; Signal Transduction ; Tamoxifen ; pharmacology

OBJECTIVETo review the experience of different surgical construction methods for hypopharyngeal cancer with cervical esophageal invasion.

METHODSFrom 1989 to 2000,forty-eight patients with advanced hypopharyngeal cancer and cervical esophageal invasion were retrospectively reviewed, including 38 males and 10 females. The median age was 54. 3 years old, ranged from 26 to 71 years old. According to UICC 1997 criteria, all the tumors were T4 stage and originated from the pyriform sinus (33), posterior pharyngeal wall (14), postcricoid area (1), there were 28 patients in cN0, 15 in cN1, 5 in cN2 and no distant metastasis. Precise preoperative evaluation was performed with computed tomography scan, barium swallow perspective and biopsy. All the patients received modified neck dissection, including both unilateral (38 patients) and bilateral (10 patients). Pharyngoesophageal defect reconstruction methods were: laryngotracheal flap in 11 patients, pectoralis major musculocutaneous flap in 13, laryngotracheal flap combined with pectoralis major musculocutaneous flap in 6, pectoralis major musculocutaneous flap combined with the split graft in 10, stomach pulling-up in 3, colon interposition in 5 patients. Total laryngectomy was carried out in 8 patients. All patients received radiotherapy postoperatively (dose 55 - 75 Gy).

RESULTSThe cervical lymph node metastasis was found in 20 patients. Pathologic findings showed that well, moderately and lower differentiated squamous cell carcinomas were 18, 24, 6 cases, respectively. The overall 3 and 5 year survival rates were 52.1% (25/48) and 27.3% (12/44), respectively. The 3 and 5 year survival rates in functionally preserved group were 65.2% (15/23) and 33.3% (7/21), while in non functionally preserved group were 40.0% (10/25) and 21.7% (5/23), respectively. Fifteen patients laryngeal functions (voice, respiration and deglutition) were completely restored and 8 patients partially restored (voice and deglutition). The decannulation rate was 65% (15/23). The complication included pharyngeal fistulas in 10 cases and splitting of chest wall in 1 cases.

CONCLUSIONSCombined therapy was the best choice for hypopharyngeal cancer with cervical esophageal invasion. The laryngeal function is preserved as far as possible. The continuity of the pharyngoesophagus was restored by pectoralis major musculocutaneous flap, laryngotracheal flap, or combined with the split graft. Stomach transposition or colon interposition was used while the defect of the esophagus was greater.

Adult ; Aged ; Carcinoma, Squamous Cell ; mortality ; pathology ; surgery ; Esophageal Neoplasms ; mortality ; secondary ; surgery ; Esophagus ; pathology ; Female ; Humans ; Hypopharyngeal Neoplasms ; mortality ; pathology ; surgery ; Lymphatic Metastasis ; Male ; Middle Aged ; Retrospective Studies ; Survival Rate

OBJECTIVETo evaluate the practical method of vacuum sealing drainage (VSD) technique combined with sural neurovascular pedicle fasciocutaneous flap to repair deep wounds in the foot near the ankle joint with exposed bone and tendons.

METHODSFrom January 2006 to January 2009, 79 patients with deep wounds in the foot near the ankle joint with exposed bone and tendons were treated by VSD technique combined with sural neurovascular pedicle fasciocutaneous flap including 58 males and 21 females with an average age of 34 years old ranging from 7 to 59 years. There were 17 cases in low 1/3 part of leg and achilles tendon, 28 in lateral malleolus and lateral dorsum of foot, 21 in medial malleolus and medial dorsum of foot, 13 in heel and pelma. Firstly the wounds were debrided and cultivated by using VSD technique, then the soft tissue defections were repaired with sural neurovascular pedicle fasciocutaneous flap.

RESULTSThe area of flap was from 6 cm x 5 cm to 18 cm x 15 cm; All patients stayed in hospital for 14 to 30 days, 18 days in average. Living flaps of all patients were followed-up from 6 months to 3 years, the flaps of 2 patients were mostly necrotic, 3 were necrotic, 5 cases appeared obstacle of venous back streaming. The others survived with no infections.

CONCLUSIONThe wound would become fresh and clean as soon as possible with VSD. The sural neurovascular pedicle fasciocutaneous flap could provide a good covering for the exposed wound. Therefore the wound healed faster with friction resistance and fine appearance. The time of hospitalization were greatly shortened after combined application.

Adolescent ; Adult ; Ankle Joint ; surgery ; Child ; Drainage ; methods ; Female ; Foot Injuries ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; methods ; Soft Tissue Injuries ; surgery ; Surgical Flaps ; Vacuum

OBJECTIVETo investigate gene mutations of epidermal growth factor receptor (EGFR) and K-ras in Chinese patients with non-small cell lung cancer (NSCLC) and its clinicopathological significance, and to analyze the correlation between these mutations and tumor response to erlotinib treatment.

METHODSMutations of exons 18, 19, 20 and 21 of the EGFR and codons 12, 13 of the K-ras in 301 cases of NSCLC were detected by PCR-amplification and gene sequencing. The relationship between the mutations and clinicopathological characteristics of the 301 patients was analyzed.

RESULTSEGFR mutations were present in 32.9% (99/301) of the samples: 3 mutation in exon 18, 59 in exon 19, 2 in exon 20, and 35 in exon 21. Mutations of K-ras were present in 4.7% (14/301) of the samples: 13 in codon 12 and 1 in codon 13. EGFR mutations were never found in tumors with K-ras mutations, suggesting a mutually exclusive relationship. EGFR mutations were more common in adenocarcinomas, non-smokers and females. Seven out of 10 erlotinib-treated patients with disease control carried EGFR mutation.

CONCLUSIONThe frequency of EGFR mutation in Chinese NSCLC patients is higher than that in Westerners, but the frequency of K-ras mutation is quite opposite. Combined detection of EGFR gene and K-ras gene mutation may help clinicians to choose patients who may gain benefit from EGFR tyrosine kinase inhibitor (EGFR-TKI) treatment, and to predict their response to erlotinib treatment and prognosis.

Adenocarcinoma ; drug therapy ; genetics ; pathology ; Adult ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; genetics ; pathology ; Carcinoma, Squamous Cell ; drug therapy ; genetics ; pathology ; Codon ; Erlotinib Hydrochloride ; Exons ; Female ; Genes, erbB-1 ; Genes, ras ; Humans ; Lung Neoplasms ; drug therapy ; genetics ; pathology ; Male ; Middle Aged ; Mutation ; Protein Kinase Inhibitors ; therapeutic use ; Proto-Oncogene Proteins ; genetics ; Proto-Oncogene Proteins p21(ras) ; Quinazolines ; therapeutic use ; Receptor, Epidermal Growth Factor ; genetics ; Sex Factors ; Smoking ; Young Adult ; ras Proteins ; genetics

OBJECTIVETo investigate the clinical and laboratory features of patient with B cell lymphoma associated hemophagocytic syndrome(B-LAHS).

METHODSThe clinical data of 10 cases of B-LAHS were retrospectively analysed and the relevant literatures were reviewed.

RESULTSThe median age of 10 cases diagnosed as B-LAHS was 55.5 (31-88) years old, and median time from attack to diagnosis was 2 months (2 weeks-4 months). The diagnosis can be made histopathologically and immunohistochemically by bone marrow biopsy. Among them 7 cases were diagnosed as large B cell lymphoma, 2 cases as mantle cell lymphoma and 1 case as small B cell lymphoma. The prominent clinical symptoms and signs were persistent fever (100%) and splenomegaly(90%), and the involvements with respiratory and digestive system were common. Another 1 case had systemic muscle pain and lactic acidosis as the first onset. Laboratory studies showed hepatic dysfunction, significantly elevated ferritin and lactate dehydrogenase, abnormal lymphocytes in peripheral blood smear, and hemophagocytosis in bone marrow smear. The FSC/SSC abnormalities of cloned B lymphoma cells were detected through flow cytometry (FCM). The complete remission (CR) was maintained in 4 cases receiving immunochemotherapy based on rituximab.

CONCLUSIONB-LAHS possesses heterogeneous clinical manifestations and rapid deterioration. Bone marrow biopsy and immunohistochemical examination can confirm the diagnosis. FCM may improve the early diagnosis of B-LAHS.