Cutaneous ulcers: An unusual manifestation of inherited thrombophilia

We report two cases with cutaneous lesions found to be associated with factor V Leiden mutation and low S protein levels. At the time of presentation, no other symptoms were reported. Histopathology of both lesions revealed the presence of widespread intravascular thrombi. The therapeutic response to oral anticoagulants in the first case was dramatic, leading to complete healing of the lesions. Inherited causes of thrombophilia manifested as cutaneous lesions, although rare, should be considered in the differential diagnosis of occlusive vasculopathy.     

Echocardiographic evidence of posteromedial hypokinesis of the left ventricle in relation to mitral regurgitation in cardiac sarcoidosis

We describe herein the case of a 49-year-old female patient with pulmonary sarcoidosis (stage II) with cardiac manifestation. This consisted of systolic dysfunction without dilatation of the left ventricle and severe mitral insufficiency, possibly due to thinning of the posteromedial left ventricular free wall, based on our echocardiographic observations.     

Soft tissue Ilizarov correction of congenital talipes equinovarus--5 to 10 years postsurgery

The aim of this study was to address concerns regarding maintenance of correction of foot deformity after soft tissue Ilizarov distraction in relapsed clubfeet. We retrospectively reviewed the results of Ilizarov surgery of 19 rigid clubfeet in 14 children. The postoperative follow-up was at least 5 years (range, 5-10 years). All patients were assessed using the International Clubfoot Study Group score. This is a comprehensive scoring system published in 2003, incorporating morphological, functional, and radiological assessments. Patient and parent satisfaction was also assessed. Using the International Clubfoot Study Group score, 14 of the 19 feet managed by the Ilizarov soft tissue distraction technique were graded good or excellent. Only 1 patient experienced recurrence of the deformity. Subjectively, 13 of the 14 children in the study were satisfied with the result of the treatment. The study confirms that the short-term good results reported previously are maintained at least 5 years after the operation. There are no other similar studies with a minimum 5-year follow-up.     

No significant improvement in the outcome of patients with Waldenström's macroglobulinemia treated over the last 25 years

The treatment of Waldenström's macroglobulinemia (WM) has changed over the last decades, mainly because of the introduction of nucleoside analogues and of rituximab while novel agents such as bortezomib have been recently introduced. We performed an analysis to investigate whether the outcome of patients with WM has improved over the last years, compared to that of patients who started treatment before new drugs became widely available, especially as part of the frontline treatment. We analyzed 345 symptomatic patients with WM: 130 who initiated treatment before and 215 who started treatment after January 1, 2000. Patients who started treatment in the latter group were older and had more often elevated beta2‐microglobulin but the other characteristics were similar between the two groups. Most patients who started treatment before January 1, 2000 were treated upfront with alkylating agent‐based regimens and most patients who started treatment after January 1, 2000 received rituximab‐based regimens as initial treatment. Objective response (63 and 59%, respectively) and median overall survival, OS, (106.5 months for Group A and is estimated at 94 months for Group B, P = 0.327) were similar. There was also no difference regarding OS or cause specific survival (CSS) in each risk group according to IPSSWM. Our observation may be explained by the indolent course of WM in several patients and by the lack of profound cytoreduction in patients with high‐risk disease. Possible differences in the 15‐ or 20‐year survival rate between the two groups may be detected with further follow‐up of these patients. Am. J. Hematol. 2011. © 2011 Wiley‐Liss, Inc.     

An autopsy case of necrotizing eosinophilic myocarditis causing left ventricular wall rupture

Acute necrotizing eosinophilic myocarditis (ANEM) is a rare entity with sudden onset and rapidly progressive course, usually leading to a fatal outcome. It is characterized by focal or widespread myocyte necrosis. The entity’s clinical presentation is quite variable, rendering the antemortem diagnosis difficult. A case of a 66-year-old woman dying suddenly, initially considered to have suffered a myocardial infarction and finally proved at autopsy to have died due to ANEM resulting in myocardial rupture, is presented. Left ventricular wall rupture is in the majority of cases, a complication of myocardial infarction and its association with acute myocarditis has been very rarely reported. The case reported herein highlights the infrequent presentation of ANEM as cardiac rupture. Myocardial rupture is associated with a high mortality rate, even if immediate surgical repair and intervention are provided.     

Safety Profile of Sequential Transcatheter Chemoembolization with DC Bead?: Results of 237 Hepatocellular Carcinoma (HCC) Patients

Introduction

Complications of chemoembolization performed with DC Bead^? loaded with doxorubicin (DEBDOX) of diameters 100?C300???m and 300?C500???m are presented in this paper. These diameters are currently the smallest available in drug-eluting technology.

Methods

Included are 237 patients who were treated with sequential DEBDOX with doxorubicin loaded at 37.5?mg/ml of DC Bead. The National Cancer Institute Common Terminology Criteria for Adverse Events (version 3.0) were used to categorize complications.

Results

Thirty-day mortality was 1.26% (3/237). Incidence of grade 5 complications was 1.26% (3/237). Overall, grade 4 complications resulted in 5.48% (13/237) (irreversible liver failure, cholecystitis). Grade 2 liver function deterioration developed in 10 patients (4.2%). Cholecystitis/grade 2 and 4 incidents were observed in 3.6?C5.06% across sessions (overall 13 patients; 5.48%). Postembolization Syndrome (PES) grade 1 or 2 was observed in up to 86.5%; however, grade 2 was observed in 25?C42.19% across treatments. Pleural effusion was seen in eight patients (overall 3.37%; grade 1 in 1.8?C3.7% across treatments; grade 3 in 0.42%). Grade 1 procedure-related laboratory pancreatitis was seen in 0.45%, and grade 2 gastrointestinal bleeding was seen in 0.84%. Procedure-associated skin erythema/grade 1 was seen in 0.84%. There was no correlation of liver failure or transient liver function deterioration with the diameter of the beads (p?=?0.25?C0.37 and p?=?0.14?C0.89, respectively). Stratifying with the diameter of the beads correlation values was: for cholecystitis (p?=?0.11?C0.96 across treatments), PES (p?=?0.35?C0.83), temporary/grade 1 elevation of liver enzymes (p?=?0.002?C0.0001), and bilirubin (p?=?0.04?C0.99).

Conclusions

DEBDOX chemoembolization is safe and small calibres do not result in increased complication rates compared with results of series using larger diameters of beads.