Recurrent Atypical Hemolytic Uremic Syndrome in Children With Acute Lymphoblastic Leukemia Undergoing Maintenance Chemotherapy.

Chemotherapy-associated myelosuppression and renal dysfunction is not uncommon during childhood acute lymphoblastic leukemia (ALL) therapy. Here we report 2 cases of atypical hemolytic uremic syndrome (aHUS) presenting with pancytopenia and renal dysfunction that developed during maintenance chemotherapy characterized by hypocomplementemia. Both cases experienced recurrence after resolution of the initial aHUS episode upon resumption of chemotherapy, raising a possible contributory role for chemotherapy in the disease pathogenesis.

Invasive Pneumococcal Disease in Patients With Sickle Cell Disease.

Background: Patients with sickle cell disease (SCD) are at risk of fatal sepsis with encapsulated bacteria, such as Streptococcus pneumoniae, because of the inherent autosplenectomy that occurs in SCD. This risk is thwarted with oral penicillin prophylaxis during the first 5 years of life, and with stringent vaccination against S. pneumoniae alongside routine childhood immunization.

Methimazole Induced Total Myeloid Aplasia with Delayed Recovery Despite Granulocyte Colony Stimulating Factor (G-CSF): Marrow Progenitor Recovery Kinetics.

An eighteen-year-old female with Graves thyrotoxicosis presented with methimazole-induced agranulocytosis and total myeloid aplasia. The bone marrow at presentation showed complete absence of myeloid precursors and striking plasmacytosis. 16 days later, myeloid precursors were still absent morphologically; however bone marrow flow cytometry and cell culture detected an improvement in myelogenesis, which was soon followed by clinical recovery of agranulocytosis.

Neonatal BO Incompatibility Is Associated With a Positive Cord Blood Direct Antiglobulin Test in Infants of Black Ethnicity.

ABO hemolytic disease of the newborn occurs almost exclusively in infants of blood group A and B who are born to group O mothers. Positive Direct Antiglobulin Test (DAT) can identify those infants who are at risk of developing the ABO hemolytic disease. Earlier studies have suggested that BO incompatibility is associated with a positive DAT in black infants.

Upper-extremity deep venous thrombosis after whole blood donation: report of three cases from a single blood center.

Background: There are two upper-extremity deep venous thrombosis (UEDVT) cases after whole blood donation reported in the English medical literature. Three additional UEDVT cases after whole blood donation were reported to our blood center within a 13-month period.

Study Design And Methods: A case study was done for each case in collaboration with a clinical physician.

Emergency department visits in children with hemophilia.

Background: The pediatric emergency department (ED) management of bleeding and other complications of hemophilia constitutes an increasingly important component of hemophilia therapy. This retrospective study examined the overall ED use by children with hemophilia in a single center, with a particular aim to investigate visits related to injury or bleeding, and those related to blood stream infection in patients with a central venous catheter (CVC).

Methods: Electronic medical records of patients with hemophilia presenting to Children's Hospital of Michigan ED were reviewed.

Provocative F wave in the diagnosis of nonspecific neurogenic-type thoracic outlet syndrome.

Objective: Thoracic outlet syndrome (TOS) is defined as a constellation of clinical symptoms caused by the entrapment of neurovascular structures (subclavian vessels and the brachial plexus) en route to the upper limb via the superior thoracic outlet. Nonspecific neurogenic TOS is not easy to diagnose because there is no investigational technique that has proven to be the diagnostic gold standard.

Design: In this study, our aim was to investigate the role of provocative F response in the diagnosis of nonspecific neurogenic TOS.

An audit on the current practice of red blood cell transfusion following elective primary hip arthroplasty.

This audit encompassing a six-month period on the current practice of red blood cell transfusion following elective primary total hip arthroplasty showed that the rate of allogeneic blood avoidance was 84.8% for preoperative autologous blood donors and 47.8% for non-donors (p<0.

Warfarin reversal emerging as the major indication for fresh frozen plasma use at a tertiary care hospital.

Because of the increase in the use of warfarin in the population in recent years, reversal of warfarin-related coagulopathy has become common in daily hospital practice. Transfusion of fresh frozen plasma (FFP) is the preferred treatment method for urgent warfarin reversal in the USA. We have undertaken a 1-month audit of FFP usage to ascertain the impact of warfarin use on the consumption of FFP.

The efficiency of gabapentin therapy in patients with lumbar spinal stenosis.

Study Design: Randomized controlled study.

Objectives: To investigate the efficacy of treatment with gabapentin on the clinical symptoms and findings in patients with lumbar spinal stenosis (LSS).

Summary Of Background Data: LSS is a syndrome resulting from the narrowing of the lumbar nerve root canal, spinal canal, and intervertebral foramen, causing compression of the spinal cord.

How do you treat bleeding disorders with desmopressin?

Desmopressin is an analog of vasopressin that exerts a substantial haemostatic effect by inducing the release of von Willebrand factor from its storage sites in endothelial cells. It has proved useful in treating or preventing bleeding episodes in patients with von Willebrand disease, haemophilia A and platelet function defects. Its efficacy in achieving a satisfactory level of haemostasis has reduced the use of blood products to treat bleeding episodes.

Vacuolated neuroblastoma cells mimicking FAB L(3) lymphoblasts in bone marrow aspirates.

Abundant cytoplasmic vacuolation of neuroblasts has been noted on bone marrow aspirate (BMA) smears of two patients with metastatic neuroblastoma. Occasional tumor cells were dispersed as individual cells as well as in clumps. These cells had basophilic cytoplasm and several nucleoli, reminiscent of L(3) lymphoblast morphology.

Defining the role of magnetic resonance imaging in unifocal bone lesions of langerhans cell histiocytosis.

Localized Langerhans cell histiocytosis (LCH) of bone often presents as a diagnostic challenge. Magnetic resonance imaging (MRI) is frequently used to better delineate most solitary bony lesions. The authors present two cases that illustrate and better define the role of MRI in the evaluation of solitary bone lesions of LCH.

Pediatric EBV-positive T-cell/histiocyte-rich large B-cell lymphoma with clonal cells in the bone marrow without overt involvement.

T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL) is a variant of large B-cell lymphoma only rarely encountered in children. Here we report the case of an 8-year-old African American boy with Epstein-Barr virus (EBV)-positive TCHRLBCL who initially presented with right submandibular, anterior cervical and supraclavicular lymphadenopathy. Cytogenetic analysis of the lymph node revealed a near-triploid karyotype with complex chromosomal aberrations.