Involvement Of Other Organs Research Articles

Asymptomatic Gastric Metastasis from Breast Carcinoma

Metastatic disease to the stomach from breast carcinoma is uncommon. Though most metastases are symptomatic with abdominal pain as the most common complaint, about 5% of metastases could be asymptomatic. We report a case of breast cancer with metastatic disease to the stomach that was asymptomatic and had near normal gastroscopy. Case report: A 56-year-old female with known history of Barrett's esophagus presented for her surveillance upper endoscopy. She denied associated abdominal pain, nausea, vomiting, odynophagia, dysphagia, or involuntary weight loss. Her past medical history was significant for right modified radical mastectomy with lymph node removal for lobular carcinoma of the breast approximately 7 years ago. This was treated with adjuvant chemotherapy and postoperative radiation. She also had history of stage II squamous cell carcinoma of the vagina approximately 4 years ago and was treated with external beam radiation along with platinum chemotherapy. Her physical examination was unrevealing. She underwent an EGD that showed short segment Barrett's esophagus, a diminutive benign appearing gastric polyp and antral erosions (Figures 1 & 2). Endoscopically there were no gastric lesions as nodules, ulcers or large polyps to suggest malignancy. Biopsies from the distal esophagus confirmed Barrett's esophagus without any dysplasia. But biopsies from gastric polyp and antrum revealed metastatic lobular carcinoma of the breast (Linitis Plastica pattern with involvement of lamina propria). Radiological work up including PET scan, MRI head and bone scan failed to show any metastatic involvement of other organs. This case illustrates lobular breast cancer metastasizing to the stomach without any symptoms and also without any specific endoscopic findings. [figure 1][figure 2]Figure 1Figure 2

Hepatocerebral Mitochondrial DNA Depletion Syndrome Caused by Deoxyguanosine Kinase (DGUOK) Mutations

Autosomal recessive mutations in deoxyguanosine kinase (DGUOK) have been identified in the hepatocerebral form of mitochondrial DNA (mtDNA) depletion syndrome. To describe the clinical spectrum of DGUOK-related mtDNA depletion syndrome in 6 children and to summarize the literature. We identified pathogenic mutations in DGUOK in 6 children with the hepatocerebral form of mtDNA depletion syndrome. We describe the clinical, neuroradiologic, histologic, and genetic features in these children. All children showed severe hepatopathy, while involvement of other organs (skeletal muscle and brain) was variable. We identified 5 novel mutations (1 of them in 2 children) and 2 previously described mutations. Three different mutations affected the initial methionine, suggesting a mutational hot spot. One of our patients underwent liver transplantation; pathologic findings revealed (in addition to diffuse hepatopathy) a hepatocellular carcinoma, implying a possible link between mtDNA depletion syndrome and tumorigenesis. We studied 12 children with infantile hepatoencephalopathies and mtDNA depletion syndrome and found pathogenic DGUOK mutations in 6, suggesting that this gene defect is a frequent but not an exclusive cause of the hepatic form of mtDNA depletion syndrome.

Perforation of the bowel from intrauterine contraceptive devices: case series and discussion

The intrauterine contraceptive device is used extensively in the female population. Serious complications are rare, but they do occur. We discuss three cases of bowel perforation caused by these devices following their perforation through the uterine wall. It is important that cases of perforation be recognised swiftly and the possibility of involvement of other organs considered.

Surgery in children with hydatid disease of the spleen

Background/Purpose Hydatid disease of the spleen is a rare disease, and it occurs in 1% to 8% of all children with hydatid disease. That difference in frequency depends on the social and economic status of the patients and the country. The aim of our study is to reveal its diagnosis and treatment. Methods From 1985 to 2004, 152 children with hydatid disease have been operated on at the Surgical Department of General and Pediatric surgery at the Medical University in Stara Zagora and Medical University in Plovdiv, Bulgaria. Of the 152 children, 15 (9.87%) had spleen localization: 6 males and 9 females, aged 10 to 18 years. A solitary cyst in the spleen was found in 10 patients, 8 children had an isolated cyst only in the spleen, 7 with involvement of other organs (5 in the liver, 2 in liver and lungs). All spleen cysts were “silent.” Abdominal sonography, computed tomography, and enzyme-linked immunosorbent assay tests proved the diagnosis. All children were operated on. Results A splenectomy was performed in 14 cases, and a cystectomy was possible in only 1 case. Patients with combined forms successfully underwent different variations of cystectomy: 6 omentoplasty, 5 capitonnages, and 4 invaginations. Conclusions Splenic involvement of hydatid disease has no specific clinical manifestation, the diagnosis is late and often leads to splenectomy. Organ-preserving procedures are possible only in cases with early diagnosis. It has to be suspected in a patient with a splenic cyst and has to be confirmed by ultrasonography and/or computed tomography, magnetic resonance imaging, and immunologic tests. The early diagnosis is a good precondition for better results.

Intestinal strongyloidiasis and hyperinfection syndrome.

In spite of recent advances with experiments on animal models, strongyloidiasis, an infection caused by the nematode parasite Strongyloides stercoralis, has still been an elusive disease. Though endemic in some developing countries, strongyloidiasis still poses a threat to the developed world. Due to the peculiar but characteristic features of autoinfection, hyperinfection syndrome involving only pulmonary and gastrointestinal systems, and disseminated infection with involvement of other organs, strongyloidiasis needs special attention by the physician, especially one serving patients in areas endemic for strongyloidiasis. Strongyloidiasis can occur without any symptoms, or as a potentially fatal hyperinfection or disseminated infection. Th2 cell-mediated immunity, humoral immunity and mucosal immunity have been shown to have protective effects against this parasitic infection especially in animal models. Any factors that suppress these mechanisms (such as intercurrent immune suppression or glucocorticoid therapy) could potentially trigger hyperinfection or disseminated infection which could be fatal. Even with the recent advances in laboratory tests, strongyloidiasis is still difficult to diagnose. But once diagnosed, the disease can be treated effectively with antihelminthic drugs like Ivermectin. This review article summarizes a case of strongyloidiasis and various aspects of strongyloidiasis, with emphasis on epidemiology, life cycle of Strongyloides stercoralis, clinical manifestations of the disease, corticosteroids and strongyloidiasis, diagnostic aspects of the disease, various host defense pathways against strongyloidiasis, and available treatment options.

Dapsone as a Potential Treatment for Cutaneous Rosai-Dorfman Disease With Neutrophilic Predominance

A 31-year-old Taiwanese woman, who from her medical history was generally well, presented with 1 slowly enlarging elevated plaque on the posterior aspect of her right shoulder. This had first been detected 3 months before the visit to our clinic. The lesion had originated as a small, tender, erythematous papule and had since grown into a large confluent plaque with satellite papules. She claimed that the lesion was increasing in size, and this was accompanied by intense itching and fever. On physical examination, there was 1 palm-sized, dusky red, elastic firm plaque on the right shoulder. The main lesion was surrounded by multiple 2to 8-mm erythematous satellite papules (Figure 1). There were no palpable cervical, supraclavicular, axillary, or inguinal lymph nodes. A systemic workup that included head, neck, and chest computed tomographic scans showed no involvement of other organs. Workups done to determine the source of her febrile episodes yielded no evidence of active infection. The only abnormal laboratory finding was polyclonal hypergammaglobulinemia found on serum protein electrophoresis. An incisional biopsy was performed. Microscopically, a dense dermal infiltrate extended from the upper dermis deep to the subcutis (Figure 2). The infiltrating cells were mainly composed of histiocytes with large vesicular nuclei. They also had abundant cytoplasm with spidery borders. Neutrophils, lymphocytes, and plasma cells were scattered between the histiocytes, and the presence of lymphocytes engulfed within the histiocytic cytoplasm, a feature called emperipolesis, was also noted. The mixed infiltration was neutrophil predominant. Immunohistochemical studies revealed histiocytes that stained positively for S100 protein and CD-68. Notably, the dermis surrounding the histiocytes was infiltrated by numerous neutrophils. Clinically and histologically, she was diagnosed as having cutaneous Rosai-Dorfman disease (CRD). Before visiting our hospital, she had been treated at a dermatologic clinic for 2 months, first with topical triamcinolone, oral prednisolone (30 mg/d), and then with oral isotretinoin (100 mg/d). However, the lesion had continued to worsen and the symptoms of pruritus and intermittent episodes of fever had progressed during the treatments. Superficial irradiation was suggested, but the patient refused owing to its potential hazards.

Clinical signs for identification of neurocysticercosis in swine naturally infected with Taenia solium

Taenia solium infection is a zoonotic disease and swine is the natural intermediate host. Till date no literatures have described clinical signs in swine indicative of brain involvement by cysticerci. In the present study we describe such clinical signs of porcine neurocysticercosis (NCC). These signs were excessive salivation, excessive blinking and tearing, and subconjunctival nodule. A total of 30 swine (18 with 2 or all 3 clinical signs and 12 without any sign) underwent magnetic resonance imaging (MRI). All 18 swine with above signs had NCC on MRI along with variable involvement of other organs that were subsequently confirmed by ex vivo MRI, necropsy and histopathology, while none of the 12 animals without any sign had NCC. As development of a porcine NCC model has proved difficult, we propose that naturally infected swine can be identified on the basis of these clinical signs and thus used as a model for further research on NCC.

Characteristics of Multiple Primary Malignancies in Renal Cell Carcinoma

Purpose: This study aimed to evaluate the incidence, nature and prognosis of multiple primary malignancies in renal cell carcinoma. Materials and Methods: From June 1995 to April 2004, we retrospectively reviewed the records of 578 patients who underwent an operation for renal cell carcinoma at Yonsei University. The incidence of other primary malignancies, in addition to the renal cell carcinoma, was determined and classified as antecedent, synchronous or metachronous. We analyzed the influence of the other primary malignancies on the prognosis, and the overall survival rates of the patients with multiple primary malignancies were compared to the remaining patients. Results: Of the 578 patients, 62 patients (10.7%) had at least one malignancy beside renal cell carcinoma. In these cases, renal cell carcinoma was commonly incidental, small or low stage (75.8%). 22 patients (35.4%) had gastrointestinal cancer and 11 patients (17.7%) had hepatobiliary cancer. The malignancies were antecedent in 25 (40.3%), synchronous in 24 (38.1%), and metachronous in 14 patients (22.2%). On the analysis of overall survival with using the log rank test, there was no statistically significant factor for the presence of other antecedent or synchronous malignancies. Conclusions: Primary malignancy associated with renal cell carcinoma in the Korean population shows a different pattern from that of Western countries. The expected survival rate of patients suffering from renal cell carcinoma with tumor involvement of other organs, if surgically treated, is similar to that of renal cell carcinoma alone, so aggressive surgical treatment of renal cell carcinoma should be performed. (Korean J Urol 2006;47:118-123) 󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏󰠏

A Patient with IgA Monoclonal Gammopathy Presenting as Myelomatous Pleural Effusion with Axillary Node Involvement

Hepatocellular carcinoma (HCC) is 3rd leading cause of cancer in Korea and the prognosis for HCC patients is poor. For assessing the present treatment outcome, this study analyzed the three-year survival rate (3-YSR) and the prognostic factors for patients with HCC in Korea. Between November 2000 and December 2003, 905 patients with HCC who were diagnosed and treated at the National Cancer Center Korea were enrolled in this study. The clinical variables, tumor characteristics and survival periods were analyzed. The mean age of all patients was 56.2+/-10.3 years and 732 (80.9%) patients were male (M:F=4.2:1). 508 (56.1%) patients died and the median survival period was 15.3 months. The overall 3-YSR of the patients with modified UICC stage I, II, III, IVa and IVb were 67.4%, 65.2%, 30.7%, 9.0% and 5.0%, respectively. The modified UICC stage could not differentiate stage I from II, and stage IVa from IVb, on the 3-YSR. The 3-YSR of the Child-Pugh class A patients with modified UICC stage I or II was 85.4% by surgical resection and it was 69.6% by transcatheter chemoembolization (TACE), respectively (P= .461), and those values for patients with stage III were 49.2% and 36.8%, respectively (P=.081). As compared with systemic chemotherapy or conservative therapy, TACE increased the survival rate more for the Child-Pugh class A patients with stage IV. The independent prognostic factors were serum AFP, portal vein thrombosis, the Child-Pugh classification and the stage of HCC. This follow-up study will be helpful in assessing the results of treatments for HCC and it will provide data for the establishment of a more effective treatment strategy.

The clinical staging of rectal cancer.

Abstract The ability of rectal digital examination to recognize significant stages of local extent and lymph node involvement in adenocarcinoma of the lower two-thirds of the rectum was investigated. Seventy patients with a palpable rectal cancer were examined by 2 or 3 out of a panel of 10 clinicians including 2 consultants and 8 registrars. A defined protocol was used and the results were recorded on a proforma. Clinical findings were compared with the results of pathological examination of the resected specimen or the final surgical assessment when the growth was not removed. In 38 patients computed tomography of the primary growth was performed and the results were also compared to the pathological findings. Four groups of cases with different degrees of extent of spread were recognized by digital examination in 67–83 per cent by consultants and in 44–78 per cent by registrars. Computed tomography correctly identified growths with extensive local spread in 89 per cent of cases, but was no more reliable than digital examination in the assessment of other degrees of spread or of lymph node involvement. Based on the results, a clinical staging system is proposed, comprising four stages of local extent as follows—stage 1: confined to rectum; stage 2: confined to rectum or slight extrarectal spread; stage 3: moderate or extensive extrarectal spread; stage 4: involvement of other organs or unresectability. Two stages of lymph node involvement are also proposed: namely, node negative and node positive. Each stage corresponds to different survival and local recurrence rates after surgery. Combined with other factors, such as level and size of tumour, histological grade and the general state of the patient, the clinical staging system may facilitate the choice of treatment. It might extend the use of major sphincter-saving procedures, indentify many patients suitable for local treatment and define those with a considerable risk of local recurrence where combined surgery and radiotherapy might be considered.

Non Hodgkin's lymphoma involving the adrenal glands and the central nervous system (CNS): a particular evolution after chemotherapy

Adrenal lymphoma is extremely rare. The prognostic depends on involvement of other organs (such as the central nervous system) responsible for lower median survival. We report the case of a 51-year-old man with non Hodgkin's Diffuse Large B Cell Lymphoma (DLBCL) involving the central nervous system (CNS) and the adrenal glands simultaneously. The endocrine exploration revealed a partial adrenal insufficiency and ruled out a pheochromocytoma. Computerized tomographic (CT) scan directed needle biopsy of the adrenal gland allowed the diagnostic of non-Hodgkin lymphoma (NHL). CNS biopsies showed similar histopathologic lesions. After aggressive polychemotherapy and methotrexate intrathecal injection, a dissociated therapeutic response was observed with a decrease of the cerebral lesion and an increase of the adrenal mass. This result may be explained by the efficacy of corticosteroid therapy on cerebral edema. The prognosis was poor with tumor infiltration of the leptomeninges and death 16 months after diagnosis.

Xanthoma Disseminatum with Hepatic Involvement in a Child

Xanthoma disseminatum is a rare, usually self-healing dermatologic disease of unknown etiology. Involvement of other organs and tissues including bone marrow, bone, and brain may be seen rarely in children. However, to date, hepatic involvement has not been reported. We describe a child with xanthoma disseminatum who had hepatic involvement, and discuss his course and treatment with steroid and azathioprine.

Hepatic Sarcoidosis Mimicking Klatskin Cholangiocarcinoma

A 57-year-old African-American man presented with progressive obstructive jaundice, pruritus, and weight loss. Investigations were notable for elevated liver canalicular enzymes with negative serologies for viral hepatitis, autoimmune, and metabolic liver diseases. Serum CA 19–9, CEA, and AFP were within normal limits. ERCP demonstrated a complex stricture of common hepatic duct (CHD) at the confluence with proximal extension into both intrahepatic ducts (Bismuth-Corlette classification IV) (Figure 1). Intraductal brushings across the stricture were negative for malignancy. MRI revealed a lesion measuring 2.5 × 2.5 × 2.8 cm encasing the CHD (Figure 1). At EUS, a 2.4-cm lesion was identified surrounding the CHD (Figure 3). Cytology from FNA of the lesion was negative for malignancy. Liver biopsy revealed mild lobular hepatitis without granuloma or fibrosis. CT scan of the chest showed a normal lung parenchyma without mediastinal lymphadenopathy. Given a lesion that was highly suspicious for Klatskin cholangiocarcinoma without evidence of metastasis, he underwent an exploratory laparotomy with right hepatic lobe resection and Roux-en-Y left hepaticojejunostomy. Final pathology revealed non-caseating granulomatous inflammation forming a 3.5 cm mass in the hilar soft tissue adjacent to CHD without any evidence of malignancy or infection, consistent with sarcoidosis. In this report, we describe a unique case of hepatic sarcoidosis presenting as obstructive jaundice due to the isolated involvement of CHD and bilateral intrahepatic ducts, mimicking Klatskin cholangiocarcinoma. No involvement of other organs was identified. To our knowledge, this is the first such case report in the literature. It highlights the great difficulty in establishing a definitive diagnosis for the etiology of a biliary stricture without performing an exploratory laparotomy despite the application of multiple pre-operative radiographic modalities and tissue sampling techniques. If hepatic sarcoidosis had been diagnosed pre-operatively, a therapeutic trial of corticosteroids might have been attempted initially.[figure 1]Figure

Renal transplant diabetic neuropathic patient with painless unseen foot damage

The diabetic patient with peripheral neuropathy and circulatory dysfunction is at a significantly increased risk of lower limb amputation. This risk is greatly increased in the patient with established end stage involvement of other organs. This case describes a limb-threatening episode in a diabetic patient with renal transplant and retinopathy who had previously undergone digital amputations to both feet.

Renal pathology in children with mitochondrial diseases

We studied renal involvement in 42 children with mitochondrial diseases (MDs). The diagnosis of MD was established by morphological, biochemical, and molecular genetic criteria. Renal disease was considered when patients had renal failure, nephrotic syndrome, Fanconi's syndrome or any symptomatic renal alteration. Mild tubular disorder was established if they had abnormal laboratory findings with no apparent clinical symptom. Renal involvement was found in 21 children (50%), of whom 8 had an apparent clinical picture and 13 a mild tubular disorder. Five patients with renal disease showed Debré-Toni-Fanconi's syndrome, 2 of them with decreased glomerular filtration rate (GFR). One case had nephrotic syndrome, another one presented decreased GFR, and the last one had a neurogenic bladder and bilateral hydronephrosis. Patients with mild renal disease showed tubular dysfunction with normal GFR. Renal involvement is frequent and present in about half of the children with MD. Thus, studies for evaluating kidney function should be performed on children with MD. Conversely, patients with tubulopathy of unknown origin or progressive renal disease should be investigated for the existence of MD, especially if associated with involvement of other organs or tissues. Southern blot analysis to search for large-scale mitochondrial DNA (mtDNA) rearrangements should be performed for patients with MD and kidney involvement.

Clinical and molecular aspects of the myotonic dystrophies: A review

Type 1 myotonic dystrophy or DM1 (Steinert's disease), which is the commonest muscular dystrophy in adults, has intrigued physicians for over a century. Unusual features, compared with other dystrophies, include myotonia, anticipation, and involvement of other organs, notably the brain, eyes, smooth muscle, cardiac conduction apparatus, and endocrine system. Morbidity is high, with a substantial mortality relating to cardiorespiratory dysfunction. More recently a second form of multisystem myotonic disorder has been recognized and variously designated as proximal myotonic myopathy (PROMM), proximal myotonic dystrophy (PDM), or DM2. For both DM1 and DM2 the molecular basis is expansion of an unstable repeat sequence in a noncoding part of a gene (DMPK in DM1 and ZNF9 in DM2). There is accumulating evidence that the basic molecular mechanism is disruption of mRNA metabolism, which has far-reaching effects on many other genes, in part through the induction of aberrant splicing, explaining the multisystemic nature of the disease. The unstable nature of the expansion provides a molecular explanation for anticipation. This review emphasizes the clinical similarities and differences between DM1 and DM2. It examines current views about the molecular basis of these disorders, and contrasts them with other repeat expansion disorders that have increasingly been recognized as a cause of neurological disease.

Escherichia Coli Endocarditis of the Aortic Valve with Formation of a Paravalvular Abscess Cavity

A 42-year-old male with a history of chronic aortic valve disease and urethra stenosis, was admitted with fever, dysuria, and vomiting. Escherichia coli (E. coli) was cultured from blood and urine. Repeated transesophageal echocardiographic studies performed during the early phase of the hospitalization did not exclude the possibility of infective endocarditis. A definite diagnosis was, however, not established until 6 weeks after admission. At that time a large paravalvular aortic abscess cavity had developed and urgent surgery was necessary. A possible explanation for the delayed diagnostic evaluation was the low level of suspicion, as the septicemia was believed to originate from the urinary tract, without involvement of other organs. The possibility of endocarditis due to E. coli should be considered, especially in subjects with underlying cardiac valve disease, despite an alternative source of septicemia and despite the rarity of this condition. Frequent echocardiographic studies are recommended since extensive tissue destruction may occur without any striking symptoms.

Chronic periaortitis: a spectrum of diseases

Chronic periaortitis includes idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis, and inflammatory abdominal aortic aneurysms. This review analyses the different aspects of the disease and highlights evolving concepts concerning its pathogenesis, diagnosis, and management. It has recently been reported that asbestos exposure is a major risk factor for idiopathic retroperitoneal fibrosis. An increasing number of studies showing an association with autoimmune diseases clearly support the hypothesis of a close link between autoimmunity and chronic periaortitis. Furthermore, various findings (eg, constitutional symptoms, the involvement of other organs, high acute-phase reactant levels) support the hypothesis that chronic periaortitis may be a manifestation of a systemic disease and challenge the well-known theory of a local immune response to antigens in atherosclerotic plaque. In addition to CT and MRI, which are the diagnostic modalities of choice, positron emission tomography may be useful in monitoring disease activity and response to therapy. Although there is a lack of prospective randomized trials, recent studies have highlighted the role of steroids, immunosuppressive agents, and tamoxifen in the medical treatment of chronic periaortitis. Chronic periaortitis is a rare disease with protean manifestations but, if correctly diagnosed, can be successfully managed. It should be approached in the setting of a systemic process, and clinicians must be aware that other organs may be affected. Its clinical course is chronic-relapsing, so a careful follow-up is essential. Further studies are needed to investigate the pathogenetic mechanisms and the most appropriate therapeutic options.

Linfoma cutâneo de células B: relato de caso

Os linfomas cutâneos são classificados em linfomas de células B ou de células T, sendo os primeiros menos freqüentes. O linfoma cutâneo de células B (LCCB) pode ser primário ou secundário, tendo o último história natural mais agressiva, com pior prognóstico. Os autores apresentam um caso de LCCB secundário com três anos de evolução, curso indolente, sem envolvimento de outros órgãos além da pele, com ótima resposta ao tratamento quimioterápico. A distinção entre LCCB primário e secundário é muito difícil de ser realizada, uma vez que eles são clínica e histopatologicamente indistinguíveis. Deve-se sempre pesquisar o acometimento de órgãos internos para então definir o prognóstico.

Prognosis of Veno-Occlusive Disease after Stem Cell Transplantation in the DF-VOD Trial. On behalf of the DF-VOD Investigators:.

Hepatic veno-occlusive disease (VOD) is a severe complication occurring in patients undergoing intensive chemotherapy and stem cell transplantation (SCT). Its therapy is based on general supportive procedures; however, even if these measures improve with time and new specific therapies are now available, chances of success continue to be scarce, and day + 100 mortality exceeds 50% in current series.Methods: In the DF-VOD trial, patients are being randomized to receive defibrotide (DF) or no treatment other than supportive and are followed-up for 100 days. Diagnosis of VOD is based on the Baltimore Criteria, and patients who do not meet them but have a biopsy or hemodinamically proven VOD are also eligible. Complete Response (CR) is defined as a bilirubin < 2 mg/dl and complete resolution of any other end-organ dysfunction. In this report, principal characteristics and the outcome of the whole cohort are described. Pre-SCT (age, gender, primary disease, conditioning, donor type) and clinical and laboratory characteristics at diagnosis of VOD (days from SCT to VOD, bilirubin and AST/ALT levels, and the presence of ascites and multiorgan failure (MOF)) were analyzed as prognostic factors by logistic regression models adjusted for treatment and other principal confounders.Results: Fifty-eight patients were randomized since October 2001, but 8 patients were excluded from the analysis because of missing data. Their principal characteristics at randomization and outcome are shown in table 1. The outcome of this cohort was found to be similar to that of Richardson's cohort of 78 patients, all treated with defibrotide, in which CR was 49% and day +100 survival was 42% (Blood 2003; 102:193a). Variables significantly associated with survival are shown in table 2.Conclusions: History of previous SCT, severe liver dysfunction denoted by high levels of bilirubin and AST/ALT, and the involvement of other organs seem to be independent risk factors for mortality in patients with VOD. The low survival in this population underlines the need of new treatment strategies. Randomization of patients in the DF-VOD trial is going on to produce clear evidence on the efficacy of defibrotide in this disease.Patients' characteristics and outcomeN = 50Gender (% males)30 (60)Age at randomization (SD)36.6 (16.1)Days from SCT to VOD (SD)13 (12)Primary disease: (%) ---Hematological malignancy47 (94)--- Other3 (6)Conditioning: (%)--- BUCY16 (32)--- CY + TBI16 (32)--- Other18 (36)Type of donor: (%)--- HLA-identical / Syngenic27 (54)--- Autologous5 (10)--- HLA-mismatched4 (8)--- Unrelated14 (28)Previous SCT (%)13 (26)Ascites24 (48)Bilirubin (mg/dl), median (range)6.0 (2.0-30.0)AST/ALT > 40 U/L (%)24 (48)MOF at diagnosis (%)29 (58)Complete Response (%)19 (38)Day + 100 survival (%)17 (34)-- Adults15/45 (33)-- Pediatrics2/5 (40)Characteristics associated with mortality in multivariate analysesN (%)RR (95% IC)PPrevious SCT13 (26)12.8 (1.14-143.7)0.0390Bil < 6 mg/dl AND AST/ALT < 40 U/L17 (34)1.00Bil ≥ 6 mg/dl OR AST/ALT ≥ 40 U/L17 (34)2.26 (0.40-12.8)0.3565Bil ≥ 6 mg/dl AND AST/ALT ≥ 40 U/L16 (32)16.0 (1.92-133.4)0.0103Hepatic + one additional organ dysfunction11 (22)2.81 (0.43-18.4)0.2811Hepatic + ≥ 2 additional organ dysfunction18 (36)10.4 (1.38-77.6)0.0231