A Case of Hemolytic Disease of the Newborn due to Di (a) Antibody.

Anti-Di(a) is a clinically significant red cell antibody known to cause hemolytic disease of the newborn. Here, we report on a case of mild hemolytic disease of the newborn caused by Di(a) antibody. The mother had three prior pregnancies with no history of blood transfusion. She delivered a preterm 35-week-old female newborn by cesarean section. The neonate developed anemia and mild icterus on postnatal day five with hemoglobin of 9500 mg/dL and total bilirubin of 10 mg/dL. The direct antiglobulin test on the neonate's red blood cells was positive. The maternal serum and an eluate from the infant RBCs were negative in routine antibody detection tests but were positive using commercially prepared Di(a+) red cells. The neonate was discharged home in stable condition following treatment with erythropoietin and phototherapy. When a newborn has a positive DAT in the absence of major blood group incompatibility or commonly detected RBC antibodies, an antibody to a low frequency antigen such as Di(a) must be considered. Further immunohematology tests are required to determine presence of the antibody and the clinician must be alerted to closely monitor the infant for signs of anemia and hemolysis.

Diagnostic Accuracy of MRI in Predicting Breast Tumor Size: Comparative Analysis of MRI vs Histopathological Assessed Breast Tumor Size.

BACKGROUND: Diagnostic accuracy of MRI in predicting breast tumor size: comparative analysis of breast tumor size byMRI vs histopathological assessment. PURPOSE: Tumor size is one of the most important factors in making a clinical and pathological assessment of breast cancer. The purpose of this study is to evaluate if MRI imaging is helpful for the surgeon in the preoperative accurate assessment of tumor size. METHODS: We retrospectively reviewed the charts of 124 patients who were diagnosed or managed at the Saint Francis /Mt. Sinai Regional Cancer Center. We then compared the preoperative MRI tumor size with the histopathological tumor size obtained at surgical resection. Tumors were divided into four histological groups: invasive ductal carcinoma (IDC), invasive lobular carcinoma (ILC), ductal carcinomain situ (DCIS), and mixed tumors (including mucinous, papillary, tubular, and apocrine breast carcinoma). MRI measurement of tumor size was compared against the reference size obtained from the microscopic measurement of the largest diameter of tumors. Concordance was defined as a difference ≤ 0.5 cm between MRI and pathology. RESULTS: A total of 124 patients with 147 breast tumors were included in our study. The mean age was 59.8 ± 12.72 years. Histologically, there were 81 IDC (55.10%), 35 DCIS (23.81%), 15 ILC (10.20%), and 16 mixed tumors (10.88%). Out of 147 tumors, 55.78% showed concordance of MRI and pathologic tumor size within 0.5 cm. MRI overestimated 31.97% and underestimated 12.24% of tumors. CONCLUSION: Breast MRI provides an additional tool for preoperative assessment of tumor size. In our study, we noted 56% concordance between MRI with pathological tumor size within 0.5 cm. Several factors, including grading of tumor, cancer type, and size, strongly affect the accuracy of MRI in the preoperative assessment of tumor size. High-grade tumor and DCIS are the two strongest negative factors resulting in overestimation of tumor size on MRI.

Operated bronchial carcinoids: clinical outcomes and long-term follow-up of a single institution series of 30 patients.

BACKGROUND: Bronchial carcinoids (BCs) are infrequent neoplasms that account for only 1% to 2% of all lung tumors. We reviewed the outcomes and long-term follow-up data of all patients diagnosed with BC and treated surgically at our institution between the years 2002-2009. PATIENTS AND METHODS: We analyzed the records of all patients with BC treated between January 1, 2002 and December 31st, 2009. The results were subsequently compared with the previously published data. RESULTS: Our records identified a total of 28 patients with typical carcinoids (TC) and two patients with atypical carcinoids (AC). Of these, 22 were women and eight were men with a median age of 62 (range, 23-91 years). About two-thirds of patients were symptomatic at presentation. Central and peripheral tumor location was encountered with equal frequency, with 63.3% of tumors being located in the right lung. Bronchoscopic biopsy revealed the diagnosis in 92.3% of cases. Twenty percent of patients underwent lung sparing procedures, 73.3% underwent lobectomies, and 6.7% had pneumonectomies. Mediastinal lymphadenectomy was performed in all patients. Two patients had positive nodal metastases, one of whom survived for only 10 months. Tumor recurrence was noted in two patients with TC (7.14%) and in one patient with AC. The overall five-year survival was 90% (27/30) for the entire cohort. CONCLUSIONS: Histological characteristics and nodal status probably represent the most important prognostic factors in persons with operated BCs. The female prevalence recorded in our cohort appears to contrast with previously reported almost equal gender distribution. The slightly lower percentage of lung-sparing procedures in our patients could be explained by their more advanced disease state, with tumor extension to more than one lung lobe.

Western variant of brain intravascular lymphoma displaying three distinct evolutive radiologic stages.

Intravascular lymphoma is a rare type of extranodal diffuse large B-cell lymphoma characterized by proliferation of clonal lymphocytes within small- and medium-sized blood vessels and a relative sparing of surrounding tissues. It commonly affects the central nervous system (CNS), but its atypical presentation often leads to a delayed diagnosis. We report a unique case of a 53-year-old man presenting with confusion and ataxic gait. The initial magnetic resonance imaging (MRI) of the brain showed multifocal plaque-like CNS lesions suggestive of multiple sclerosis. His condition worsened rapidly, accompanied by persistent low-grade fever and further alteration in mental status. Follow-up MRI studies suggested new parenchymal brain lesions consistent with multiple evolving embolic strokes and subsequently with brain infarcts. Biopsy showed intravascular lymphomatous brain involvement. His condition continued to deteriorate, resulting in multiorgan failure and demise. To the best of our knowledge, these clear-cut MRI stages of brain intravascular lymphoma have not been previously reported in the scientific literature. Our findings are important as the diagnosis intravascular lymphoma is commonly made postmortem, given its rapidly progressive course and lack of typical symptomatology.

Skin necrosis: a rare complication of protein S deficiency.

Hereditary protein S deficiency is an autosomal dominant disorder leading to recurrent venous thrombosis and, less commonly, to arterial thrombosis. Cases of skin necrosis have been documented in patients with protein C or S deficiency while being treated with warfarin. We describe herein a patient with protein S deficiency who developed significant skin necrosis without being exposed to warfarin. She had a protracted clinical course resulting in gangrene and transmetatarsal amputation. Recognition of this rare complication and an earlyhematology referral may prevent dismal outcomes in patients with protein S deficiency.

Gastrointestinal stromal tumor of small intestine and synchronous bilateral papillary renal cell carcinoma.

Association of gastrointestinal stromal tumors (GISTs) with other primary malignant neoplasms has previously been reported. In addition, coexistence of unilateral renal cell cancer and a GIST of the stomach has been documented in the literature. We report herein a unique case of a GIST of the small intestine and bilateral papillary renal cell carcinomas in a patient presenting with melena and dizziness. Literature shows that GIST arising from the small intestine is the most common location of GIST accompanied by a second primary neoplasm. However, a unique feature in our GIST patient is the presence of synchronous (bilateral) papillary renal cell carcinomas.

Ischemic stroke as a presenting feature of Wegener's granulomatosis.

Wegener's granulomatosis (WG) is an autoimmune disease of unknown origin characterized by necrotizing granuloma of both upper and lower respiratory tracts and glomerulonephritis. Neurological manifestations in the central nervous system (CNS) and peripheral nervous system (PNS) are a rare but well-documented feature of WG in the literature. We describe a 51-old-male who presented with complaints of slurred speech and left-sided weakness, along with bilateral pulmonary nodules on initial chest x-ray, which were associated with pulmonary symptoms in the past. A repeated computed tomography (CT) scan showed ischemia in the distribution of the right-middle cerebral artery. An open lung biopsy showed vasculitis with necrosis of a vessel wall, with a multinucleated giant cell, confirming the diagnosis of WG. The patient was successfully treated with cyclophosphamide and oral prednisone and he has continued in remission for one year.

Immune alterations in malignant melanoma and current immunotherapy concepts.

INTRODUCTION: Malignant melanoma is a highly aggressive, immunogenic tumor that has the ability to modulate the immune system to its own advantage. Patients with melanoma present numerous cellular immune defects and cytokine abnormalities, all leading to suppression of the host anti-tumor immune response. Innovative treatment strategies can be achieved through employing our knowledge of the melanoma-induced immune alterations. AREAS COVERED: The authors review comprehensively the immune abnormalities in individuals with melanoma, and provide a summary of currently available melanoma immunotherapy agents that are currently on the market or undergoing clinical trials. EXPERT OPINION: Ipilimumab, a monoclonal antibody directed against the CTLA-4, is one of the current forefront treatment strategies in malignant melanoma. Novel immunomodulating agents have shown clear activity in patients with malignant melanoma. These include anti-PD-1 and anti-PD-1 ligand antibodies that may soon become important items in the anti-melanoma armamentarium. Combinations of different immunotherapy agents, between themselves or with other agents, are currently being studied in an attempt to further enhance the antineoplastic effect in patients with malignant melanoma.

Primary respiratory failure due to inclusion body myositis: think outside the box.

Inclusion body myositis features a slowly progressive inflammatory myopathy characterized by progressive proximal muscle weakness in the lower extremities, followed by proximal, upper-extremity weakness and later involvement of distal muscles groups. Although the most severely affected muscles are those of the limbs, the disease can also involve the respiratory, cardiovascular and gastrointestinal system muscles as well. We describe a unique patient who presented with acute hypercapnic hypoxic respiratory failure secondary to inclusion body myositis. Our patient required mechanical ventilation but responded to corticosteroid therapy. The diagnosis was delayed in part because of the slowly progressive course of the disease and the fact that an extensive investigation had not disclosed a cause. We postulate that muscle biopsy may be warranted in select patients suffering from a protracted muscle weakness.

Adult biphasic pulmonary blastoma.

Pulmonary blastoma is a rare malignant tumor, histologically resembling the fetal lung. Since its first description in 1945, only about 200 cases have been reported worldwide. This tumor predominantly affects children, but has also been reported in adults with a peak incidence in the fourth decade of life. Pulmonary blastoma has a variable clinical course that cannot be determined by its histological appearance. We report a 51-year-old patient with a large biphasic pulmonary blastoma who was treated with surgical excision. The patient remains disease-free eleven months postoperatively. As relapse rates are high in patients with large biphasic (type 2) tumors, the patient is being monitored closely. Although a rare occurrence after the age of 20, pulmonary blastoma should remain in the differential diagnosis of a lung mass in an adult.

An unusual presentation of a carcinoid tumor of the common bile duct.

CONTEXT: Carcinoid tumors arising from the bile ducts account for only a small fraction of biliary tract cancers. CASE REPORT: We report herein a 42-year-old man with a carcinoid tumor of the common bile duct. He presented with abdominal pain, bloating and dyspepsia. Clinicolaboratory and imaging studies suggested a probable obstructive common bile duct lesion. The patient underwent an endoscopic retrograde cholangiopancreatography with a stent placement in view of common bile duct decompression. Persistence of symptoms prompted a laparotomy and pancreaticoduodenectomy that revealed a well-differentiated carcinoid tumor originating in the common bile duct. CONCLUSION: Clinician's familiarity with the unusual sites of origin of neuroendocrine tumors and/or atypical presentation of such tumors may facilitate their early recognition and allow for a timely intervention.

Acute Wernicke encephalopathy and sensorineural hearing loss complicating bariatric surgery.

Health-care professionals must be aware of the mandatory vitamin supplementation in patients status post bariatric surgery. A recent increase in the number of gastric bypass surgeries in US has been associated with a proportional increase in Wernicke encephalopathy reports. Subtle or atypical neurologic features are not uncommon. Our report is of a female patient with acute Wernicke encephalopathy accompanied by sensorineural hearing loss six weeks after bariatric surgery. The patient had only a partial recovery of her neurologic symptoms eightweeks after vigorous therapy for this condition. Symptomatic thiamine (vitamin B1) and vitamin B12 deficiencies are particularly concerning effects of bariatric procedures, as neurologic and cognitive deficits may be long lasting or even permanent despite aggressive replacement therapy.

Anaphylactic reactions with intravenous vitamin K: lessons from the bedside.

Vitamin K is used as a correction factor to avert the risk of bleeding due to warfarin overdose. Among the reported complications of this therapy, the most serious one is the anaphylactic cardiovascular collapse caused by intravenous infusion of the drug. We report herein a patient with over-anticoagulation from warfarin use and a subcapsular renal bleeding who developed a fatal anaphylactic shock after vitamin K administration via slow intravenous infusion. Vigilance with the intravenous formulation of this agent should always be sought in order to prevent fatal outcomes, especially in patients with severe comorbidities.