Spectrum Of Vascular Lesions Research Articles

Postmastectomy-Postirradiation Atypical Vascular Lesion of the Skin: Report of 2 Cases

The spectrum of vascular lesions developing in breast or chest wall skin following lumpectomy or mastectomy and radiation is wide and ranges from atypical vascular lesions with a benign clinical behaviour to frankly malignant, angiosarcoma ranging histologically from well to poorly differentiated variety. Postmastectomy-postirradiation atypical vascular lesions (AVLs) are rare and develop in the skin adjacent to the mastectomy scar. About hundred cases have been reported in the literature so far. AVLs have also been reported in patients after breast conservation surgery within the breast parenchyma or in the skin around the scar. The incidence appears to be rising. The exact reason for this is not known. The newer modalities of radiation therapy may be contributory to the pathogenesis. More studies have to be done in this area to prove the causal relationship. We are reporting the cases of 2 patients with carcinoma of breast who developed postmastectomy-postirradiation atypical vascular lesions. The cases were received in our department within a 6-month period.

The Impact of Hydroxyurea Therapy on the Prevalence of Retinopathy in a Pediatric Sickle Cell Cohort

Abstract 1057 Introduction:Approximately 15–17% of children with HbSS and 11% of those with HbSβ0thalassemia (HbSβ0thal) develop sickle retinopathy (SR) by 18 years of age. Sickle retinopathy is a spectrum of vascular lesions that insidiously evolve as a result of ischemia from vaso-occlusion of capillary and precapillary arterioles in the peripheral retina. These lesions are usually clinically silent but can progress to spontaneous vitreous hemorrhage or retinal detachment and lead to blindness. Therefore, periodic screening retinal exams are recommended for children and adults with sickle cell disease (SCD). Prior reports from Jamaica identified low fetal hemoglobin (HbF) concentration as a potential risk factor for retinopathy in HbSS patients. Hydroxyurea (HU), an increasingly utilized treatment in children with HbSS/HbSB0thal, increases %HbF levels. However, there are no reports describing the effects of HU therapy on the prevalence of retinopathy in children with SCD. In our institution, all children with SCD are referred to ophthalmology for a screening dilated fundoscopic exam (DFE) beginning at 10 years of age and then every 2 years. Objective:Define the prevalence of retinopathy in children with HbSS/HbSβ0thal and analyze the effects of treatment with HU and %HbF levels on the prevalence of retinopathy. Methods:After IRB approval, a retrospective chart review was performed on children with HbSS/HbSβ0thal who received a DFE without fluorescein angiography at St Jude Children’s Research Hospital between 2003 and 2010. Children who received chronic transfusion therapy or hematopoietic stem cell transplantation were excluded from analysis. Records of DFEs were reviewed for a diagnosis of non-proliferative or proliferative SR. Laboratory data were collected at multiple time points from patients’ electronic medical records, including: prior to initiation of HU, following two years of therapy with HU, and immediately prior to diagnosis of retinopathy. Time of observation was defined as birth to diagnosis of SR or to date of last DFE. Summary statistics are reported and the HU and non-HU groups are compared with the Wilcoxon-Mann-Whitney Exact test. Results:Of 182 children referred to ophthalmology, 142 (78%; 126 HbSS, 16 HbSβ0thal) received an initial DFE at a median age of 13.1 ± 3.3 years (range: 1.6–18.7 years). The median number of DFEs per patient was 1.7 ± 1.4 (range: 1–12) during the time of observation. Sickle retinopathy was diagnosed in 9.9% (14/142) of children, at an average age of 15 ± 2.5 years. Of the children who received a DFE, 63% (90/142) had initiated HU therapy at an average age of 9.8 ±4.8 years. The average age at initial DFE was not different in patients receiving HU (13.1 ± 3.2 years) and those who did not (13 ± 3.5 years). The total number of DFEs performed in both groups was also similar (1 ± 1.2 in HU-treated group vs. 1 ± 1.6 in untreated group). Retinopathy was diagnosed in 7.8% (7/90) of children who received HU and 13.5% (7/52) of children who did not (p=0.27). The median age at diagnosis of SR was similar for patients treated with HU (15.4 ± 2.5 years, range: 11.3–18.2 years) compared to untreated children (14.6 ± 2.6 years, range: 10.8–17.3 years; p=0.6). Among patients receiving HU, those who developed retinopathy started treatment at a later age, however the difference was not statistically significant (12.2 ± 5 years vs. 9.3 ± 4.5 years; p=0.23). Among the HU treated group, %HbF following 2 years of HU therapy was similar in those who developed SR (16.1 ± 7.7%) and those who did not (20.0 ± 6.8%; p=0.24). Conclusion:Although the average age at diagnosis with retinopathy in these children with HbSS/HbSβ0thal was similar to previously reported untreated pediatric cohorts, the overall prevalence of retinopathy was lower. Treatment with HU did not affect the development of retinopathy nor did %HbF levels in those treated with HU. More studies are necessary to analyze the effects of HU treatment on development of SR. In the meantime, continued adherence to screening guidelines for ophthalmology evaluations are necessary for children with HbSS and HbSβ0thal being treated with hydroxyurea. Disclosures:Off Label Use: Use of hydroxyurea in children with sickle cell anemia.

Canine visceral leishmaniosis: a remarkable histopathological picture of one case reported from Brazil

This report describes a remarkable histopathological presentation of a symptomatic dog naturally infected with Leishmania ( Leishmania) chagasi from Brazil. An intense inflammatory granulomatous reaction was observed in the liver and spleen associated with hypertrophy and hyperplasia of the mononuclear system (the classical histopathological picture of the disease). In addition, a spectrum of vascular lesions was observed in many organs. However, we did not find parasites (amastigotes of Leishmania) in any skin fragments of the ear, nose and or abdominal tissue. In fact, this animal had severe clinical signs, showed parasites in many organs, but no parasites in the skin. It appears that the presence or absence of parasites in the skin is not a good indicator of parasites in other organs or vice versa.

Malignant peripheral nerve sheath tumour with vascular differentiation: a report of four cases

Four cases of malignant peripheral nerve sheath tumour showing vascular differentiation are described. One case was associated with neurofibromatosis 1 and contained angiosarcomatous, cartilaginous and rhabdomyoblastic elements. The other cases occurred in patients without neurofibromatosis and showed a spectrum of vascular lesions ranging from lobulated, haemangioma-like structures to angiosarcoma. These are the first recorded examples of this phenomenon not associated with neurofibromatosis. Immunohistochemical examination demonstrated the endothelial nature of the lesions in all cases and revealed cells positive for alpha-smooth muscle actin, probably pericytes, closely apposed to the endothelium.

RENAL HOMOTRANSPLANTATION; LATE FUNCTION AND COMPLICATIONS.

Fifty-one patients were treated with homografts obtained from both related and nonrelated volunteer donors in the interval from November 24, 1962, to February 10, 1964. Thirty-three of the 51 patients lived for 4 or more months postoperatively, and 30 of these are still living on June 10, 1964. The three deaths which occurred after 4 months were due to a combination of gastrointestinal hemorrhage and sepsis in one; to a cerebrovascular accident of undetermined etiology in the second; and to uncontrolled late rejection in the third, which was first diagnosed 229 days after operation, 66 days before death. The two homografts examined at autopsy after 207 and 295 days were both enlarged and showed glomerular hypertrophy, tubular atrophy, mild cellular infiltrations, marked interstitial fibrosis, and destruction of peritubular capillaries. The case that died in uncontrolled rejection also showed interstitial edema and a spectrum of vascular lesions. Late nonfatal rejection was observed in five other cases 112 to 300 days postoperatively. In all these cases, and in the unsuccessfully treated one as well, certain consistent features were present. There had always been a preceding alteration in steroid dose, prednisone having been discontinued in five of the six patients from 16 to 154 days previously. Progress of the rejection was slow, and the proper diagnosis depended most strongly upon demonstration of a gradual fall in creatinine clearance. Elevations in blood pressure, transplant wound tenderness, fluid accumulation, alopecia, polyarthritis, fever, malaise, and anorexia were all observed. Azotemia was a late finding. The diagnosis of late rejection is one of exclusion. Pyelonephritis, vascular anastomotic failure, and ureteral obstruction are first ruled out. The last possibility is a particularly important one since 4 of the 33 patients who lived for 4 months or longer have been shown to have partial ureteral obstruction which required secondary repair. In one case, there was strong evidence that the stricture resulted from healing of an earlier ureteral rejection. If diagnosed promptly, late homograft rejection can be reversed. The crucial step in the treatment of rejection is the resumption or increase of steroid dosage. In addition, local irradiation to the graft and intravenous actinomycin C are both of use as emergency therapy. The chronic administration of azathioprine has thus far appeared to be very well-tolerated by those patients who are receiving only this drug, although its ultimate toxicity will take years to determine. The superimposition of steroid therapy, in those patients whose homograft function cannot be otherwise maintained, adds a serious risk. Gastrointestinal hemorrhages, infections, aseptic bone necrosis, potentiation of hypertension, and severe obesity have all been observed. The course of five patients who received preliminary thymectomy and who survived more than 4 months is compared to that of the other recipients who did not receive this additional operation. One of the patients who had thymic excision died after 207 days of nonrenal complications. The other four had steroids discontinued in 9 months or less, and none have had any evidence of delayed rejection 14 to 18 months after operation. The 6 examples of late rejection all occurred in the other 28 long-surviving patients who did not have removal of the thymus gland. The possible role of thymectomy in influencing these late events remains to be determined. Renal function ranged from adequate to essentially normal levels in all patients who survived longer than 4 months, except the one who died of uncontrolled late rejection. In four cases, in which late renal function was tested at a time when rejection was not present, the results in paired donors and recipients were almost identical, suggesting hypertrophy in the homograft as well as in the donor’s residual kidney. These results indicate that considerably more than half the patients who are treated with renal homotransplantation can be brought into a chronic stage, even when the statistics are unfavorably altered by the use of a substantial number of nonrelated donors as was the case in the present series. The data also indicate that overoptimism must be avoided in assessing the future role of this form of therapy since it is quite evident that many adverse factors still exist in the residual group of living patients, and that life-threatening late complications are going to be common. These include toxicity from drugs, especially if indefinite steroid therapy is required; late rejection; urologic complications; and subtle subclinical progression of pathologic alterations in the homograft.