Resolution of Hematocolpos in Adolescents Affected with Graft vs Host Disease

Background

Bone marrow transplantation is a lifesaving procedure for a range of serious benign or malignant hematological conditions. A proportion of patients, however, will develop graft vs host disease (GVHD), acute or chronic, with serious long-term sequalae.

角膜异物致眼部损伤的处理体会

目的：总结角膜异物致眼部损伤的处理方法。方法：本组358例患者，先滴盐酸奥布卡因做表面麻醉，在裂隙灯显微镜下，用氯霉素滴眼液浸湿无菌棉签轻轻拭去。对于嵌入角膜表面的异物，局部滴1％盐酸奥布卡因后，用1ml的注射器针头将异物剔除。结果：本组病人角膜异物中一次性取净339例，经2次除锈取净19例；无一例发生术后感染及角膜穿孔等并发症。结论：角膜异物处理需仔细认真操作，避免对局部组织的损伤，预防感染。     

Follicular mucinosis occurring after bone marrow transplantation in a patient with acute lymphoblastic leukemia.

Follicular mucinosis (FM) is characterized histologically by mucinous degeneration of the outer root sheath of the hair follicle and sebaceous gland, accompanied by inflammatory infiltrate. It can occur as a primary idiopathic disorder or in association with benign or malignant diseases, most notably mycosis fungoides. In addition, it also can be found incidentally on histology. We describe an unusual case of follicular mucinosis in a 19-year-old man with acute lymphoblastic leukemia (ALL). One month after bone marrow transplantation, he developed cutaneous graft-versus-host disease (GVHD) involving the palms and soles, which was followed 12 days later by the appearance of multiple erythematous follicular papules and plaques on his face, auricles, and postauricular area. Biopsy of follicular plaque revealed changes of follicular mucinosis without evidence of graft-versus-host disease or leukemia cutis. The follicular rash was associated with prominent peripheral eosinophilia. The rash and eosinophilia resolved after 2 and 4 weeks, respectively. In conclusion, we report a case of FM occurring as a transient reaction during the course of cutaneous GVHD following bone marrow transplantation for ALL. Awareness of this condition may avoid undue concern that the rash might represent a manifestation of GVHD, cutaneous relapse of the hematological malignancy, or a drug allergy.     

Incidence of corneal abrasions during pelvic reconstructive surgery

Objectives

To compare the incidence of corneal abrasions after robotic/laparoscopic sacral colpopexies versus vaginal apical suspensions, and to determine risk factors associated with the development of corneal abrasions.

Study design

This retrospective cohort study included all women undergoing robotic/laparoscopic sacral colpopexy or vaginal apical suspensions over a 5-year period. The incidence of corneal abrasions was compared between groups and statistical analysis was performed.

Results

5/216 (2.3%) patients developed corneal abrasions in the sacral colpopexy group compared to 1/332 (0.3%) in the vaginal group (p = 0.04). Eye protection was more frequently documented in the sacral colpopexy group compared to the vaginal group (98.6% vs. 83.4%, p < 0.001). Women in the sacral colpopexy group were younger, with longer operating times, more intravenous fluids, and lower estimated blood loss. Risk factors for corneal abrasion could not be identified due to the low number of patients with corneal abrasions.

Conclusion

More corneal abrasions occurred with laparoscopic and robotic sacral colpopexy compared to vaginal apical suspension procedures. Risk factors could not be identified in this study.     

Wound healing patterns following perforation sustained during laser in situ keratomileusis.

BACKGROUND AND PURPOSE: Corneal perforation during laser in situ keratomileusis (LASIK) may interfere with flap adhesion and wound healing. The purpose of this study was to investigate wound healing patterns following corneal perforation sustained during LASIK in rabbits. METHODS: Forty-two pigmented rabbit eyes underwent LASIK surgery with 5.0-mm excimer laser treatment under the corneal flap. Animals were divided into two groups: group I (n = 19) underwent the regular LASIK procedure with -10.0 D treatment, without perforation; in group II (n = 23), the cornea was perforated with the excimer laser. Treatment was discontinued once perforation was observed, and the corneal flap was replaced without sutures. Slit-lamp biomicroscopy, photography, and scatterometry were performed preoperatively and at 1 and 2 days, 1 week, and weekly up to 1 month, 2 months, and 3 months postoperatively. Animals were killed at 1 day, 1 week, 1 month, and 3 months postoperatively and processed for light microscopic, electron microscopic, and immunohistochemical examinations. RESULTS: In group I, the corneas remained clear throughout the experiment. In all eyes, the interface was not readily discernable clinically or histologically. Corneal wound healing was accompanied by minimal cell infiltration. Epithelial hyperplasia at the flap edge was noted at 1 week. Myofibroblast activation was found at the epithelial wedge where there was an epithelial basement membrane break. In group II, the anterior chamber was shallow with no iris incarceration at the end of surgery. The corneas were clear (n = 6) or showed mild to moderate edema (n = 12). Corneal edema peaked at 3.6 +/- 5.0 days and subsided thereafter. Corneal wounds healed similarly to those in group I except at the perforation site. The break in Descemet's membrane and endothelium was covered with a fibrin plug on day 1, which resolved thereafter. There was no statistically significant difference in the incidence of postoperative infection (p = 1.0) or flap displacement (p = 0.69) rates between the two treatment groups. The scatterometry index peaked at 2 to 3 weeks postoperatively and was significantly higher in group II than in group I (p < 0.001). CONCLUSIONS: Although corneal perforation during LASIK surgery may interfere with immediate postoperative flap adhesion, corneal wound healing following LASIK perforation may be similar to that after an uncomplicated LASIK procedure.     

Hematopoietic stem cell transplantation in Taiwanese children with primary immunodeficiency.

BACKGROUND AND PURPOSE: Since 1968 it has been known that hematopoietic stem cell transplantation (HSCT) can ameliorate primary immunodeficiencies, but data on the long-term efficacy of this treatment in Taiwan are limited. This study analyzed the outcome of HSCT and the immune reconstruction in 10 children with primary immunodeficiencies in Taiwan. METHODS: We retrospectively analyzed the outcome of HSCT in 10 children with primary immunodeficiencies between 1986 and 2002. The primary immunodeficiencies in these children included severe combined immunodeficiency (SCID) in 4, Wiskott-Aldrich syndrome (WAS) in 4, Chediak-Higashi syndrome (CHS) in 1, and leukocyte adhesion deficiency (LAD) in 1. The conditioning protocols included busulfan and cyclophosphamide in 2 patients with SCID, 3 patients with WAS, 1 patient with LAD and 1 patient with CHS. Anti-thymocyte globulin was given to only 1 patient with WAS and no conditioning therapy was given in 2 SCID patients. Graft-versus-host disease (GVHD) prophylaxis with cyclosporine (CsA) and methotrexate was prescribed in 6 children, CsA alone in 1, and CsA and T-cell depletion in 1. RESULTS: Six patients were cured and 1 improved during a follow-up period from 3.5 years to 13 years after transplantation. Three patients died of severe sepsis. Three patients developed acute GVHD, which was grade 2 in 2 patients, and grade 3 in 1. Veno-occlusive disease developed in 1 patient and chronic GVHD with contracture of joints in 1. CONCLUSIONS: Our results support the benefits of allo-HSCT in children with primary immunodeficiencies. However, HSCT should be performed as soon as possible before severe infection develops.     

The many faces of multivisceral transplantation

The transplantation of multiple abdominal viscera, including liver-duodenum-pancreas, liver-stomach-duodenum-pancreas and liver-intestine, is being performed with increasing frequency and success. These procedures and other variations are derived from a seldom used multivisceral operation in which all of the foregoing organs are transplanted en bloc. It is described herein how the full multivisceral transplantation and its less extensive derivatives are based on the same principles of procurement, preservation and postoperative management. With all of these multiple organ permutations and with intestinal transplantation alone, management is complicated by inclusion in the grafts of a large lymphoreticular component that is capable of causing graft versus host disease (GVHD). Because of a systematic error in therapeutic philosophy, past efforts have been directed at altering or damaging the lymphoreticular cells by pretreatment of the donor or of the organs with drugs, irradiation or other means. From recent observations, the alternative approach is suggested of keeping these lymphoid depots intact, which then become the site of two way cell traffic after transplantation. With the use of powerful immunosuppression, such as that provided with FK 506, the donor lymphoreticular cells can circulate in the recipient without causing clinical GVHD, and the lymphoreticular cells in the graft become those of the recipient (local chimerism) without causing rejection. Even with avoidance of rejection and GVHD, metabolic interrelations between the grafted organs, and also between the graft organs and retained recipient viscera can affect the fate of the individual transplanted organs or retained recipient organs. The best delineated of these metabolic influences are mediated by the endogenous splanchnic hepatotrophic factors, of which insulin has been the most completely studied. An understanding of these various immunologic and nonimmunologic factors combined with more potent immunosuppression that is now available is sure to stimulate efforts at transplantation of abdominal organs and particularly of the hollow viscera that have resisted such clinical efforts.     

The management of severe vaginal obstruction from genital chronic graft-versus-host disease: diagnosis, surgical technique and follow-up

AIM: Chronic graft-versus-host disease (GVHD) is one of the most important systemic late-onset complications of haematopoietic stem cell transplantation. Gynaecological manifestations are considered relatively rare, and involve lower genital tract skin and mucosa, causing vulvar scarring, vaginal stenosis, affecting the patients' sexual life, and leading to more serious complications as haematocolpos, haematometra and abscesses. Genital GVHD can be treated with topical therapy when mild to moderate disease is present. Surgery is indicated in advanced and complicated cases to restore normal anatomy. The aim of this study is to propose a standard approach for the management of such condition when medical therapy is not effective. METHODS: From May 2000 to January 2002, 8 patients suffering from genital chronic GVHD were operated in our institution. We describe clinical and sonographic presurgical assessment, simple or ultrasonographic guided surgical technique, postsurgical treatment and follow-up. RESULTS: Surgery was completely successful in restoring genital anatomy in all the cases. Mean follow-up was of 17 months (6 to 38 months). Two patients early discontinued the postsurgical treatment. At 1 month complete vaginal patency was found in 6 cases, weak partial adhesions in 1 case, while 1 patient refused follow-up. Eventually, complete vaginal patency was maintained in 2 cases, and partial adhesions were found in 5 cases. CONCLUSIONS: The combined use of clinical examination and endosonography provides a precise assessment of the level of the obstruction. The surgical technique here described is feasible and successful in restoring normal anatomy, while long term results seem related to the compliance towards the postsurgical treatment.     

A case report of unusual complication of myomatous uterus in pregnancy: spontaneous perforation of myoma after red degeneration

An unusual complication of myomatous uterus in pregnancy is presented. It shows spontaneous perforation of a myoma after red degeneration, presenting as an acute abdomen. To our knowledge spontaneous perforation of a necrotising leiomyoma has not been reported earlier. A review of the literature is given.     

Severe vaginal chronic graft-versus-host disease (GVHD): two cases with late onset and literature review

Female genital graft-versus-host disease (GVHD) occurs after allogenic stem cell transplantation (SCT) in 25% of long-term survivors. To date, 28 severe cases with hematocolpos requiring surgery have been documented. We report two cases of severe gynecological GVHD. Although most of the vaginal GVH disease cases occurred within two years after oncologic treatment, our two cases highlight the possibility of a very long onset. We also confirmed that hormonal replacement therapy does not protect against synechia and that corticoid treatment has a good therapeutic value in recurrence after surgery. In conclusion, women with SCT experience a high risk of vaginal GVHD that could impair quality of life and sexual function. These cases pinpointed the importance of very long-term gynecological follow-up in order to diagnose early symptoms. To date, only early therapy with local corticoid application might reduce symptoms and development of severe genital GVHD. We support systematic use of topical corticoid therapy in severe cases even after surgery because of the high incidence of recurrence.     

Needle laparoscopy in patients with suspected bowel adhesions.

Suspected bowel adhesions are generally considered to be a contraindication to laparoscopy. Among 32 patients who were refused laparoscopy for that reason, only 1 patient was found to have adhesions at laparotomy. To attempt preoperative diagnosis of intraabdominal adhesions, needle laparoscopy was performed in 21 patients using a 1.7-mm endoscope. In 20 of the patients it was possible to establish the presence or absence of adhesions and to determine the feasibility of laparoscopic sterilization. This preliminary evaluation suggests that needle laparoscopy may avert bowel perforation in some instances and may permit laparoscopic tubal sterilization to be performed in some women who would otherwise, because of multiple previous operations, be denied laparoscopy.     

Allogeneic stem cell transplantation for patients with high-risk myelodysplastic syndrome.

Allogeneic stem cell transplantation (allo-SCT) is the only treatment with curative potential for patients with myelodysplastic syndrome (MDS). From June 1986 to April 1997, we treated 12 patients with primary MDS (5 men, 7 women, median age, 36.5 years) by allo-SCT. All patients had one or more of the following poor prognostic factors: intermediate-2 or high-risk categories according to the International Prognostic Scoring System; disease progression during follow-up; heavy transfusion requirements and recurrent infections. The median duration from diagnosis of MDS to allo-SCT was 6 months. The preconditioning regimen included total body irradiation combined with either high-dose cytarabine (n = 6), high-dose cyclophosphamide (n = 4), or other regimens (n = 2). Ten patients received bone marrow transplantations and two patients received peripheral blood stem cell transplantations. Prophylaxis for graft-versus-host disease (GVHD) consisted of standard cyclosporin and short-course methotrexate. Acute GVHD of grade 2 or above occurred in 10 patients, while chronic GVHD occurred in seven of the nine patients who survived longer than 6 months after allo-SCT. With a median follow-up of 50 months, all nine patients with human leukocyte antigen (HLA)-matched sibling donors survived. One patient had a relapse 6 months after transplantation and achieved complete remission again with low-dose cytarabine therapy. The three patients receiving allo-SCT from unrelated or HLA-mismatched donors died of grade 3 to 4 acute GVHD and infection within 5 months after transplantation. The estimated disease-free survival at 4 years was 67% (95% confidence interval, 40-93%), and the overall survival was 75% (95% confidence interval, 50-99%). Our data suggest that allo-SCT should be considered early in the clinical course for young MDS patients with a poor prognosis and a matched sibling donor.     

母亲供髓移植治疗儿童复发非霍奇金病1例并文献复习

目的 探讨母亲供髓异基因造血干细胞移植(Allo HSCT)治疗恶性复发非霍奇金淋巴瘤的疗效和供体造血干细胞来源问题。 方法 2004年1月，第一军医大学南方医院儿科与海军总医院儿科给1例8岁男性淋巴母细胞淋巴瘤(LBL)患儿移植了母亲来源的HLA不全相合、ABO血型不合的骨髓造血干细胞。预处理选用阿糖胞苷、足叶乙苷、环磷酰胺和全身照射。预防移植物抗宿主病(GVHD)采用兔抗人T 淋巴细胞免疫球蛋白、环孢菌素A、甲氨蝶呤和CD25单克隆抗体。移植有核细胞数8.92×108/kg，单个核细胞数为1.89×108/kg，CD34细胞数为1.37×106/kg，CD3细胞数为32.9×107/kg。 结果 粒细胞绝对数>0.5×109/L的天数是移植后15d(+15d)，血小板>30×109/L的天数是+20d，+27d采用荧光原位杂交性染色体检测显示99%为供者型。+37d受者血型由O型转变为供者血型B型。患儿于+19d出现Ⅰ度急性GVHD，给予激素冲击后，口服小剂量维持治疗，渐消退，随访1年余，未发生慢性GVHD。 结论 母亲来源的骨髓造血干细胞移植对LBL有根治性治疗作用，而且在一定程度上解决造血干细胞来源问题。     

Endoscopic sphincterotomy and endoscopic fulguration in the management of adenoma of the papilla of Vater

Adenoma of the papilla of Vater is a rare premalignant lesion usually treated by local surgical resection. Five patients with adenomas of the papilla, presenting with obstructive jaundice, underwent endoscopic sphincterotomy with reversal of hepatic biochemistry to normal. Thereafter, four patients underwent local surgical excision. The adenoma recurred in all four patients within six to 18 months after the operation. The recurrent adenomas were eradicated by diathermic fulguration through the endoscope and were not found during a 12 to 24 month follow-up period. Snare polypectomy was performed upon one patient (who refused surgical treatment) who had a flat, broad-based adenoma, with an incomplete resection of the adenoma. This patient had adenocarcinoma of the head of the pancreas develop 40 months later. We conclude that endoscopic sphincterotomy is effective for biliary decompression of adenoma of the papilla of Vater presenting with acute obstructive jaundice. Endoscopic snare polypectomy of the adenoma combined with fulguration may be used as an alternative to surgical treatment in high risk patients.     

Multiple recurrent papillomas of the breast: case report

We report the case of a patient presenting multiple recurrent papillomas of the breast. This mild mammary pathology, developed at the expense of the galactophoric ducts, recurred four times, in a nodular way, in spite of in sano surgical resection. The retrospective study of the cell proliferation and the DNA quantification of the cells constitutive of the papillomas, on several samples, underlined evolutive lesions, which could be predictive of a malignant degeneration. This observation allows us to discuss the management of this rare mild mammary pathology, which can sometimes lead to invasive breast carcinoma.     

Cyclosporine-induced encephalopathy in a patient with relapsed acute myeloid leukemia treated with unrelated allogeneic bone marrow transplantation.

Cyclosporine (CSP) is the most frequently used immunosuppressive agent for prevention of graft versus host disease (GVHD) in allogeneic bone marrow transplantation (BMT). Some adverse effects such as hepatic and renal toxicity have been frequently encountered, but central nervous system (CNS) toxicity caused by CSP is rare. We report an adult male patient with acute myeloid leukemia who developed CSP-induced encephalopathy under treatment for allogeneic BMT from an unrelated donor. Methotrexate and CSP were used for GVHD prophylaxis. Leukocyte and platelet engraftment were successfully achieved on days 21 and 24 after BMT, respectively. Abrupt onset of mental confusion and disorientation occurred on day 25, followed by a generalized tonic clonic seizure and consciousness disturbance. The whole blood CSP level was 160.65 ng/mL. Magnetic resonance (MR) imaging revealed high signal intensities in the bilateral occipital lobes with predominant involvement of the cortical areas. The patient recovered from the CNS toxicity, but with slight memory impairment, 6 days after CSP was discontinued. When patients receiving CSP treatment for allogeneic BMT develop mental confusion, consciousness disturbance, or seizure, CSP-induced CNS toxicity should be taken into consideration.     

Genital Manifestation of Graft-vs.-Host Disease: A Series of Case Reports

IntroductionAfter hematopoietic stem cell transplantation (HSCT), many patients present genital graft-vs.-host disease (GVHD) that can culminate with sexual problems, which are poorly dimensioned.AimWe hope to draw attention to the need to perform genital biopsy to diagnose genital GVHD, and thus to call attention to the need to incorporate careful attention to sexual health in the treatment of these patients.MethodsFive allogeneic stem cell transplant recipients complaining of coital pain after HSCT were clinically diagnosed for genital GVHD. Genital biopsies were given for histological analysis, and microphotographs of the corresponding marked field in the slide were taken. Specimens were evaluated by the site pathologist and then sent to a reference pathologist, each blinded to the histological findings. A literature search was performed in PubMed/MEDLINE (1966–2009) for cross-sectional and cohort studies or trials related to genital GVHD. Expert opinions peer reviews and case reports were also considered.Main Outcome MeasuresHSCT, genital GVHD, genital biopsy.ResultsThe biopsy showed evidence of dilated apoptotic cells in the basal layer and detachment of the epithelial lining of the mucosa, hyalinization and thickening of collagen fibers, capillary ectasia, and mononuclear inflammatory infiltrate of the submucosa. Three patients presented vulval lesion such as leucoplasia and ulcer on the large lip. Histological analyses showed evidence of epithelial hyperplasia and influx of inflammatory cells to the epithelial surface, intercellular edema and spongiosis, apoptotic bodies on the basal layer of the epithelium, spongiosis, and nuclear vacuolization. A common treatment based on corticotherapy resulted in complete remission of coetaneous or mucous genital lesions in all five patients.ConclusionGenital biopsy is important to differentially diagnose GVHD and secondary symptoms due to hypoestrogenism. Prevention is the most important step in controlling the evolution GVHD in the vagina to prevent vaginal obstruction and sexual dysfunction. da Silva Lara LA, de Andrade JM, Mauad LMQ, Ferrarese SR, Marana HRC, Tiezzi DG, and de Sá Rosa e Silva ACJ. Genital manifestation of graft-vs.-host disease: A series of case reports.     

Treatment of pulp floor and stripping perforation by mineral trioxide aggregate.

Mineral trioxide aggregate (MTA) has been widely used to repair various kinds of tooth perforations, but its use for obturation of the entire root canal has not been reported. We report two cases of tooth perforation successfully repaired with MTA. The first patient was a 78-year-old male with calcified canal and pulp floor perforation in the left maxillary first premolar. After bypass of the calcified palatal canal, both buccal and palatal canals were filled with gutta percha, and the pulp floor perforation was repaired with MTA. Clinical success with no evident radicular lesion was found at the 18-month follow-up. The second patient was a 51-year-old female with a stripping perforation in a C-shaped root canal of the right mandibular second molar detected after removal of a post. Following root canal debridement and calcium hydroxide therapy to control inflammation at the stripping perforation site, apical and furcation bone healing were observed by radiographic examination. The stripping perforation was repaired by obturation of the entire C-shaped root canal with MTA. Observation at the 9-month follow-up revealed bone healing without any clinical symptoms and signs. These cases suggest that MTA is an alternative root canal obturation material for treatment of stripping perforation in a C-shaped root canal and for repair of pulp floor perforation.     

Use of T-cell antibodies for donor dosaging in a canine model of in utero hematopoietic stem cell transplantation

AIM: Microchimerism following canine in utero hematopoietic stem cell transplantation (IUHSCT) development of T-cell dosing regimens. OBJECTIVE: To investigate the use of anti-T-cell antibodies for cell dosing of the donor graft in a canine model of IUHSCT. STUDY DESIGN: Canine IUHSCT was performed by ultrasound-guided intraperitoneal injection in days 35-38 of fetal canines with CD34(+) cells at doses of 4.5 x 10(8) to 1.3 x 10(9) cells/kg and T cells (CD3(+) CD5(+)) at doses of 8 x 10(6) to 8.8 x 10(8) cells/kg. Postnatal studies included tissue histology and polymerase chain reaction-based chimerism analysis. RESULTS: Term survival was 86-100%. Microchimerism (0-2%) was detected in five of eight recipients in multiple tissues. Histopathology revealed no evidence of graft-versus-host disease (GVHD). CONCLUSION: Canine IUHSCT is a useful model to investigate the role of donor T cells in engraftment and GVHD. IUHSCT at early gestational ages with high doses of donor T cells in the graft yields microchimerism in multiple tissues without GVHD.     

Matched-unrelated-donor bone marrow transplantation for children with leukemia.

BACKGROUND AND PURPOSE: This report describes the results of matched-unrelated-donor transplant for leukemia or myelodysplasia in the first 23 recipient children at a single medical center in Taiwan. METHODS: Between August 1994 and February 2003, 23 consecutive children with leukemia or myelodysplasia underwent matched-unrelated-donor bone marrow transplantation (BMT). The preparative regimen consisted of fractionated total body irradiation and cyclophosphamide in 6 patients; busulfan in combination with etoposide and cyclophosphamide in 4 patients who received cranial irradiation before transplantation; and busulfan and cyclophosphamide in 13 patients. RESULTS: Engraftment was achieved in 91.3% of cases. Acute graft-versus-host disease (GVHD) occurred in 18 of 21 patients who engrafted (85.7%). Event-free survival for all patients was 24.46 +/- 9.24%. The 12 children with standard-risk disease had better event-free survival than the 11 children with high-risk disease (46.88 +/- 15.03% vs 0%, p < or = 0.001). CONCLUSIONS: The major obstacles to successful matched-unrelated-donor BMT are acute GVHD, relapse and infection. Early transplantation and patient selection, prophylactic and therapeutic maneuvers for GVHD, as well as appropriate donor selection and virus prophylaxis may improve the results.