Kasabach-Merritt syndrome of the leg associated with osteolysis or Gorham sign

INTRODUCTION: Kasabach-Merritt syndrome and Gorham's sign are two uncommon and severe, sometimes life-threatening, complications in infants with vascular lesions. Their association has been described in rare cases. CASE REPORT: An infant with a vast congenital angiomatous lesion including an extensive lymphatic component, developed active regional osteolysis then suddenly suffered disseminated intravascular coagulation of the leg. Medical treatment was unsatisfactory. After unsuccessful use of low molecular weight heparin, pentoxifyllin and alpha interferon, amputation of the leg was required to avoid a fatal outcome. DISCUSSION: Kasabach-Merritt syndrome does not develop on classic immature hemangiomas, despite some contradictory statements in the literature. In our case, a complex tumor developed in association with a lymphatic malformation. The association of Kasabach-Merritt syndrome with osteolysis (Gorham's sign) does not appear to be fortuitous. Therapeutic management of these severe complications is difficult and requires case by case analysis.     

Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon

Giant vascular neoplasms in neonates generally require aggressive medical or surgical therapy for treatment of complications. Steroids, chemotherapy, embolization, radiation, and surgery have all been used with short-term beneficial and sometimes unknown long-term side effects. A new modality of treatment, alpha-interferon, has recently been described. The majority of hemangiomas in children involute by 8 years of age. Occasionally, hemangiomas can endanger vital structures and are associated with a consumption coagulopathy and thrombocytopenia (Kasabach-Merritt Syndrome). These hemangiomas occasionally do not respond to steroids, radiation therapy, cytotoxic drugs, or embolization. The mortality rates approach 50% in nonresponders. Alpha-interferon has been used in these children with life-threatening complications of hemangiomas with relief of symptoms. This case illustrates the potential use of alpha-interferon in the management of giant hemangiomas in children. This emerging form of biological therapy avoids the risks of radiation therapy, embolization, and surgery with only minimal side effects.     

Surface-induced mild hypothermia anesthesia for hepatectomy in a patient with a giant hemangioma of the liver (Kasabach-Merritt syndrome)

A 63-year-old woman presenting with thrombocytepenia and signs of intravascular coagulation (prothronbin time, 59%: FDP, 100 micrograms.ml-1) due to a giant hemangioma of the liver (Kasabach-Merritt syndrome) was scheduled for the resection of the right lobe of the liver. In order to protect the liver on occlusion of the right hepatic artery and portal vein, we induced mild hypothermia technique with vasodilation and surface cooling by convecting warming device together with hepatoprotective agents of PGE1 and ulinastatin. Severe, acute massive bleeding occurred due to the injury of the middle hepatic vein and from the resected surface of the liver. Her rectal temperature was 31.9 degrees C during massive bleeding. Her hemoglobin decreased to 3.9 g.dl-1. Total estimated blood loss was 22,000 ml. The weight of the resected liver was 2.5 kg. The maximum postoperative levels of T-Bil and GOT were 2.47 mg.dl-1 and 171 IU.l-1, respectively. The liver seemed to have been well preserved and no other complications were observed. The hemangioma was completely removed by excision of the right lobe of the liver. Subsequently, all coagulation parameters returned to normal, indicating a complete reversibility of the coagulopathy. Surface-induced mild hypothermia is a useful and valuable method for protecting the liver during severe massive bleeding.     

Lipoprotein changes in patients with chronic hepatitis C treated with interferon-alpha

OBJECTIVE: The aim of this study was to evaluate the effects of interferon-alpha therapy on the lipid profile of patients with chronic hepatitis C. METHODS: In 36 consecutive patients with chronic hepatitis C, fasting lipoproteins were evaluated prospectively at baseline, 1, 3 and 6 months during interferon-alpha therapy and 3 months after the end of treatment. RESULTS: During interferon-alpha therapy, there was a progressive increase in total and very low density lipoprotein (VLDL)-triglycerides, VLDL-cholesterol and a sustained raise in apolipoprotein (apo) B. In parallel, there was a reduction in high density lipoprotein (HDL)-cholesterol and apo A1 levels. In contrast, total and low density lipoprotein (LDL)-cholesterol and lipoprotein (a) levels remained essentially unchanged during interferon-alpha therapy. Three patients developed chylomicronemia, two of them with severe hypertriglyceridemia, although none of them presented with pancreatitis. Chylomicronemia and severe hypertriglyceridemia were more common in patients with basal triglycerides above 200 mg/dl. Nineteen patients responded to interferon-alpha therapy, but their lipid profile did nor differ from that of nonresponders. Three months after the end of interferon-alpha therapy lipid changes subsided, although VLDL and HDL-cholesterol and apo B did not reach basal levels. CONCLUSION: In patients with chronic hepatitis C, interferon-alpha therapy is associated with an increase of total and VLDL-triglycerides, VLDL-cholesterol and apo B, and a decline of HDL-cholesterol and apo A1. The development of chylomicronemia and severe hypertriglyceridemia in some cases makes mandatory a close monitoring of triglycerides during interferon-alpha therapy, particularly among patients with increased triglycerides at baseline.     

Cytology of pigmented renal cell carcinoma. A case report

We used a new combined chemo- (COP/ABVD), radiation and interferon-alpha (10 x 10(6) IU s.c. 3x per week/12 months) therapy regimen to treat severe multicentric Castleman's disease (CD) complicated by relapsing Behcet's disease (BD) manifestations. More than 16 years after diagnosis of CD the patient remains in very good clinical condition, with remission of all CD and BD manifestations 13 months after discontinuation of the interferon-alpha treatment. In addition, our clinicopathological, immunohistological and virological data suggest a pathogenetic link between CD and BD via activation of pre-existing BD-specific plasma cells due to CD-related HHV8-induced overexpression of interleukin-6.     

The diagnostic value of symptoms and signs in childhood abdominal pain

Amiodarone is a benzofuran derivative with a chemical structure similar to thyroxine. Originally introduced to treat angina pectoris, amiodarone was found to have antiarrhythmic properties, and in 1985, was approved in the United States for treatment of life-threatening ventricular arrhythmias. It is now used for various ventricular and supraventricular arrhythmias refractory to conventional first-line medications, and as a result, side effects have been observed with increased frequency. The most severe and potentially life-threatening of these side effects is the development of pulmonary toxicity. Typically, amiodarone pulmonary toxicity (APT) is manifested by acute pneumonitis and chronic fibrosis. Amiodarone-associated hemoptysis (AAH) is a rare occurrence. The authors describe a case of AAH successfully treated with cessation of drug and steroid therapy.     

Strongyloidiasis associated with nephrotic syndrome

We report a nephrotic syndrome patient with eosinophilia who developed ileus, epigastralgia and malabsorption due to strongyloidiasis which became symptomatic by steroid therapy. The patient was then treated with thiabendazole and recovered. A percutaneous renal biopsy revealed minimal change nephrotic syndrome. This renal injury may be brought on by severe infection of Strongyloides stercoralis. It is important to rule out strongyloidiasis prior to corticosteroid therapy to patients from eosinophilia endemic areas.     

Experimental approaches for studies of particulate matter: the case for stronger consideration of dosimetric and exposure issues

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Conservative treatment of hemangiomas in infancy and childhood with interferon-alpha 2a

Hemangioma is the most common tumor of infancy. Most hemangiomas proliferate for 8 to 18 months and then slowly regress over the next 5 to 8 years. So far, steroids have been the established means of treatment, but there have been some reports of the use of interferon-alpha 2a (IFN-A2a) in such tumors. To evaluate the efficacy of IFN-A2a in treating giant hemangiomas in infancy and childhood, we administered it to ten patients aged 4 h to 6.5 years with life-threatening hemangiomas in various parts of the body. The dose used was 3 MU/m2 body surface area subcutaneously every second day. All children had previously received corticosteroids for 2 to 5 weeks without obvious success. The results were satisfactory: five patients had total or almost total regression; the rest have had partial regression while treatment is still in progress. A flu-like syndrome with fever, rhinitis, etc. accompanied the injections in some patients. The symptoms were controlled with paracetamol as necessary. Mild, transient leukopenia occurred in some children as well, but subsided after cessation of therapy for 1 week. IFN-A2a appears to be a promising and well-tolerated element in the treatment of life-threatening hemangiomas in infancy and childhood.     

Aplastic anemia in neonatal lupus erythematosus

OBJECTIVE: To describe an infant with neonatal lupus erythematosus associated with aplastic anemia. SETTING: The pediatric department in a tertiary-care hospital. INTERVENTIONS: Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy. MEASUREMENTS/MAIN RESULTS: The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8+ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died. CONCLUSIONS: Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.     

Breast cancer in older women

The American Geriatric Society currently recommends screening mammography for women up to 85 years of age whose life expectancy is three years or longer. The value of clinical breast examinations in older women needs further study. Total mastectomy and partial mastectomy with postoperative radiation therapy yield similar results in localized breast cancer. Postoperative irradiation may be avoided in women with small tumors (2.5 cm or less in diameter) who have undergone quadrantectomy. Lymph node dissection is important for tumor staging but significantly increases the risks and morbidity of surgery. Lymph node mapping may obviate the need for lymphadenectomy in many older women. Adjuvant hormonal therapy for at least two years appears to be beneficial in all women with hormone-receptor-rich tumors. Adjuvant chemotherapy is indicated in women with lymph node involvement or high-risk tumors with no lymph node involvement. Unless life-threatening metastases are present, hormonal therapy is the first approach to metastatic cancer. Chemotherapy is indicated if endocrine therapy is unsuccessful or life-threatening metastases are present. Most chemotherapy regimens appear to be well tolerated, even by women over 70 years of age. Special treatment should be employed for metastases to tumor sanctuaries (i.e., brain, eyes), the long bones, the spine and the chest wall.     

Prenatal diagnosis of Klippel-Trénaunay-Weber syndrome as a cause for in utero heart failure and severe postnatal sequelae

Ultrasound examination led to prenatal diagnosis of Klippel-Trénaunay-Weber syndrome complicated by early fetal congestive heart failure. The postnatal course was complicated by the Kasabach-Merritt syndrome of thrombocytopenia due to platelet consumption within the haemangioma. There was need for neonatal cardiopulmonary resuscitation and limb amputation.     

Developments in the treatment of patients with chronic myeloid leukemia

In the treatment of chronic myelocytic leukaemia (CML) allogeneic bone marrow transplantation (BMT) from a sibling donor is the treatment of choice in patients younger than 50 years; the overall 5-year survival is about 60%. Donor lymphocyte infusion without chemotherapy may serve as an efficacious therapy for recurrence of CML after allogeneic BMT. Application of allogeneic BMT from a matched unrelated donor is increasing, but the outcome is still inferior to that of sibling BMT, largely due to more severe and more frequent complications. Treatment with interferon-alpha has been shown to prolong the overall median survival, but especially in a subgroup of patients (about 15%) who achieve a major cytogenic response. Combination of interferon-alpha and low-dose cytarabine leads to increased survival and a better cytogenic response than interferon-alpha alone. Current investigations are aimed at further improving survival and cytogenic response by using more intensive chemotherapy, such as high-dose cytarabine, followed by interferon-alpha maintenance therapy.     

Priapism following splenectomy in an unstable hemoglobin: hemoglobin Olmsted beta 141 (H19) Leu-->Arg

Approximately 10-20% of the reported patients with acute febrile neutrophilic dermatosis (Sweet's syndrome) have an associated neoplasm. Oral findings of Sweet's syndrome are rarely reported, and no cases in patients with oral cancer have been reported to date. This report describes the clinico- and histopathological findings of Sweet's syndrome in a patient with oral cancer, treated with radiotherapy. After 10 fractions of external beam radiotherapy, treatment was interrupted because of severe oral mucositis which extended beyond the radiation fields. Two days later the patient developed multiple tender skin lesions and the diagnosis Sweet's syndrome was made. Skin and oral lesions resolved without additional treatment and did not recur upon resuming radiotherapy. As suggested in previous case reports, tumour antigens might play a role in the development of Sweet's syndrome. In this case, irradiation therapy may also have been a trigger for this syndrome.     

A review of allergic granulomatous angitis (AGA) (Churg-Strauss syndrome)

We reported clinical features of 11 patients with Churg-Strauss syndrome (CSS). Subjects included 2 males and 9 females with a mean age of 56.3 year-old. The age of the onset of bronchial asthma was high, and most of the patients had been suffered from frequent and severe asthmatic attacks during the period preceding the vasculitis. At the onset of the disease, patients showed a variety of vasculitic symptoms, including fever and neuropathy. Peripheral blood eosinophilia was universally seen. Three patients died of heart failure or central nerve involvement. Vasculitis ameliorated in 8 cases following steroid treatment. Discontinuing maintenance therapy with low-dose steroid resulted in a relapse in only one case. The significance and indication for long-term maintenance therapy should be further clarified. Based on these findings and previous publications, the clinical characteristics of CSS in Japan are discussed.     

Life-threatening transient neonatal Beh?et's disease

This case report describes transient neonatal Beh?et's disease, with life-threatening complications in the neonate. Male Baby R developed blood-streaked diarrhoea 5 days after birth, followed by recurrent severe scarring orogenital ulceration and vasculitic skin lesions. In this sixth week of life, he developed stridor leading to a respiratory arrest and necessitating assisted ventilation. No infective cause was isolated. Baby R responded well to i.v. and subsequent oral steroid therapy. At 8 weeks old he had fully recovered and remains well. Baby R's mother was not previously known to have Beh?et's disease. During the pregnancy, she began to suffer orogenital ulceration, associated with skin lesions typical of Beh?et's disease. Mild orogenital ulceration has become recurrent.     

Ruthenium-106 brachytherapy for peripheral retinal capillary hemangioma

OBJECTIVE: This study aimed to evaluate the efficacy and safety of ruthenium-106 brachytherapy of large peripheral retinal capillary hemangiomas. DESIGN: A retrospective case series. PARTICIPANTS: In 25 eyes of 24 patients, peripheral capillary retinal hemangiomas were treated. INTERVENTION: Brachytherapy using 106-ruthenium/106-rhodium plaques was performed. MAIN OUTCOME MEASURES: Eyes were reviewed for hemangioma regression after brachytherapy, occurrence of retinal detachment, requirement of additional vitreoretinal surgery, final visual outcome, and final retinal status. RESULTS: Preoperative mean visual acuity of all eyes treated was 20/60, mean hemangioma diameter was 3.8 mm, corresponding to approximately 2 disc diameters. In 14 eyes, the retina was attached before surgery, 8 eyes showed an exudative detachment, and 3 eyes showed a traction detachment. Fifteen patients had definite von Hippel-Lindau syndrome. Twenty-three of 25 hemangiomas could be destroyed by single brachytherapy. In 16 eyes, a favorable outcome could be achieved. In nine eyes, outcome was unfavorable, characterized by a severe drop in visual acuity, a persisting exudative retinal detachment, or a recurrent traction detachment. In one eye requiring repeated brachytherapy, irradiation retinopathy occurred. Hemangiomas up to a size of approximately 5.0 mm without preoperative exudative detachment could be treated safely by brachytherapy, whereas a larger hemangioma size or a pre-existing exudative retinal detachment predisposed to an unfavorable outcome. CONCLUSION: Solitary peripheral retinal hemangioma can be ablated effectively by ruthenium-106 brachytherapy. A favorable outcome can be expected if the hemangioma diameter is 5.0 mm or smaller and if there is no preoperative exudative retinal detachment.     

Differential sensitivity of hepatitis C virus quasispecies to interferon-alpha therapy

The quasispecies nature of hepatitis C virus was investigated in a patient with chronic hepatitis C virus infection who underwent interferon-alpha therapy. The hepatitis C virus E2/NS1 region was amplified and cloned, and multiple clones were sequenced before and after interferon-alpha therapy. The hepatitis C virus quasispecies can be grouped into three groups by phylogenetic tree analysis. Quasispecies from all the groups were present before interferon-alpha therapy. However, only group 3 remained after interferon-alpha therapy. In addition, only group 3 hepatitis C virus quasispecies were present during the early biochemical relapse. These data indicate that various groups of hepatitis C virus quasispecies may have different sensitivity to interferon-alpha.     

Efficacy of proton therapy in circumscribed choroidal hemangiomas associated with serious retinal detachment

OBJECTIVE: The purpose of the study is to evaluate the efficacy and safety of proton therapy in complicated circumscribed choroidal hemangiomas. DESIGN: The study design was a retrospective review. PARTICIPANTS: Studied were 13 patients (13 eyes) who had circumscribed choroidal hemangioma associated with serous retinal detachment. Of these, four eyes previously underwent laser unsuccessfully. INTERVENTION: Proton therapy including a total dose of 30 Cobalt-Gray-Equivalent was administered to each eye. MAIN OUTCOME MEASURES: Patients were controlled for initial and final best-corrected visual acuity, slit-lamp examination, intraocular pressure, fundus examination, fluorescein angiography, and tumor thickness on B-scan ultrasonography. RESULTS: The mean follow-up period was 26 months (range, 9-48 months). Retinal reattachment was obtained in all cases after a mean period of 52 days. The tumor height decreased in all cases. Visual acuity improved to two lines or more in eight eyes (62%) and reached 20/200 or more in nine eyes (69%). No radiation complication was detected during follow-up. CONCLUSIONS: Proton radiation seems to be effective and safe in the management of choroidal hemangioma associated with serous retinal detachment. It may be useful when photocoagulation can not be performed.     

Stevens-Johnson syndrome caused by a health drink (Eberu) containing ophiopogonis tuber

Stevens-Johnson syndrome is considered to be a severe type of erythema exsudativum multiforme. It is characterized by erythema with bullous and eroded lesions of skin and mucous membranes. We report a case of Steven-Johnson syndrome following consumption of a health drink containing ophiopogonis tuber. A 66-year-old female took an O.T.C. health drink for fever. The next morning, she noted erythema and swelling of her face, neck, and chest. She started to develop bullous and eroded lesions on the skin of her entire body and the mucous membranes of her oral cavity, conjunctiva, and cornea, and she became feverish. She had high degrees of corneal erosion and liver dysfunction. Skin biopsy showed diffuse necrosis of the epidermis. After admission to the hospital, steroid pulse therapy (1000 mg/day of methylprednisolone sodium succinate) was continued for 5 days. The health drink induced a positive drug lymphocyte stimulation test (DLST) and patch test. A challenge test was done with a one hundredth dose, and it was positive. We did patch tests with all components of the drink and found that Mai-Meu-Dong-Tang (ophiopogonis) alone was positive at 72 hours. There is no previous report of Stevens-Johnson syndrome caused by a health drink or Mai-Meu-Dong-Tang. Even though it is a health drink, we should be aware of the possibility of a severe reaction.