Ruth Padmore

Hemolytic transfusion reactions after administration of intravenous immune (gamma) globulin : a case series analysis

BACKGROUND: This case series summarizes our observations of hemolytic reactions after the administration of large amounts of intravenous immune (gamma) globulin (IVIG).

Hemolysis upon intravenous immunoglobulin transfusion.

Intravenous immunoglobulin (IVIG) is a mainstay of therapy in many disorders. An uncommon adverse side effect is IVIG-related hemolysis. Risk factors for IVIG-related hemolysis have been identified, including high dose IVIG given to non-O blood group recipients with an underlying inflammatory state. IVIG-related hemolysis has been linked to anti-A and anti-B hemagglutinins in the IVIG preparations and may involve both IgG and complement mediated hemolysis. A two-hit mechanism with threshold effect is proposed for IVIG-related hemolysis. Strategies exist to minimize or avoid IVIG-related hemolysis.

Possible mechanisms for intravenous immunoglobulin–associated hemolysis: clues obtained from review of clinical case reports

Intravenous immunoglobulin (IVIG) is an efficacious treatment modality for a number of conditions and is usually well tolerated with few reports of serious adverse events; however, the administration of IVIG may occasionally result in clinically significant hemolysis.

Prothrombin complex concentrate for the urgent reversal of warfarin. Assessment of a standard dosing protocol

BACKGROUND Octaplex®, a six factor prothrombin complex concentrate (PCC), has recently been approved for use in Canada. The optimal dose of Octaplex has yet to be established and our study was designed to monitor the efficacy of a low standard dose. STUDY DESIGN AND METHODS Patients on warfarin treatment in need of urgent reversal for bleeding, invasive procedures or surgery were given a standard dose of 40 ml (1000 IU FIX, 14 IU/kg). We conducted a retrospective chart review of 231 patients. RESULTS Patients given concurrent frozen plasma (FP) for reversal were eliminated from the study. Overall, 150 patients were reviewed and divided into three groups: (1) non-CNS bleeders, (2) CNS bleeders, and (3) non-bleeders. Correction of INR to 1.5 or less was achieved to the same extent in each group. Patients with active bleeding had the least successful bleeding cessation and patients with intracranial bleeding had the most dismal outcome compared to non-intracranial bleeders. CONCLUSIONS Our data suggests that Octaplex, when given as a low standard dose is effective at INR reversal with 76% of our patients correcting to an INR of 1.5 or less. It appears that this dose is sufficient for non-bleeding patients. Bleeding patients may benefit most from a dose increase to achieve more complete reversal and patients with intracranial bleeding should achieve more complete reversal within 2h of presentation.

The effect of patient‐controlled analgesia on coadministered red blood cells

BACKGROUND:  Patient‐controlled analgesia (PCA) provides effective pain control. The possibility of administrating opioids in the same line as red blood cells (RBCs) for patients with poor venous access has been entertained. The literature on this approach is not extensive, but generally cautionary.

Isolated pleural PTLD after cardiac transplantation

UNLABELLED PREAMBLE: Epstein-Barr virus infection (EBV) and immunosuppression promote emergence of posttransplant lymphoproliferative disorders (PTLD) in patients undergoing organ transplantation. OBJECTIVE We report a case of PTLD confined to the pleura. FINDINGS The patient was a 62-year-old male who had undergone cardiac transplant in 1993 for ischemic heart disease. Seven years later, he presented with dyspnea and bilateral pleural effusions. The CT scan revealed left sided pleural base thickening. The cytology of the pleural fluid and fine needle aspirate of the pleura was both suggestive of PTLD. However, the tissue submitted for ancillary studies did not contain the diagnostic material. A clinical decision was made to withdraw immunosuppressive therapy and start rituximab. His clinical course was complicated by Pneumocystis carinii pneumonia and he died 4 months after the diagnosis of PTLD. Autopsy revealed bilateral pleural effusions with pleural nodules involving the visceral and parietal pleura of both lungs. Immunohistochemistry demonstrated B cell lineage with kappa/lambda ratio of 1. PCR studies done on the pleural nodules (postmortem specimen) revealed the presence of EBV DNA and absence of human herpes virus 8 (HHV8) DNA. In situ hybridization revealed positive staining for EBV RNA within the neoplasm. CONCLUSION Pleural-based PTLD is rare. Cytology in conjunction with immunophenotyping and molecular studies can be useful for a definitive diagnosis. In our case, cytology sample was suggestive of PTLD. PCR studies performed on the antemortem specimen confirmed the presence of monoclonal IgH gene rearrangement, while the postmortem specimen revealed oligoclonal IgH gene rearrangement. The change from monoclonal to oligoclonal IgH gene rearrangement suggests reversion of monoclonal to polyclonal PTLD following rituximab and CHOP therapy. We also demonstrated EBV DNA and RNA in the tumor nodules, supporting EBV-induced PTLD.

Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression

AbstractLangerhans cell sarcoma is a rare and aggressive high grade hematopoietic neoplasm with a dismal prognosis. It has a unique morphological and immunotypic profile with a CD1a/ langerin/S100 + phenotype. T cell lineage markers except for CD4 in Langerhans cell sarcoma have not been documented previously. We report a case of 86 year-old male of Caucasian descent who presented with an enlarging right neck mass over 2 months with an underlying unknown cause of anemia. Computed tomography scan of the neck, chest and abdomen revealed generalized lymphadenopathy and mild splenomegaly suspicious for lymphoma. Diagnostic core biopsy performed on right neck mass revealed a possible T cell lymphoma with expression of T cell lineage specific marker CD3 but conclusive diagnosis could not be made due to insufficient core biopsy sample. Further excisional biopsy performed on a left inguinal node showed a hematopoietic neoplasm with features of Langerhans cell sarcoma with a focal cytoplasmic CD3 expression in 30-40% of the tumor cells. PCR for T cell receptor (TCR) gene rearrangement failed to demonstrate a clonal gene rearrangement in the tumor cells arguing against a T cell lineage transdifferentiation, suggesting an aberrant CD3 expression. To the best of our knowledge, this case represents the first report of Langerhans cell sarcoma with an aberrant cytoplasmic CD3 expression.Virtual slideshttp://www.diagnosticpathology.diagnomx.eu/vs/2065486371761991

Critical values in Hematology

Critical values are life‐threatening results that require immediate notification to the patients healthcare provider. Accreditation bodies require laboratories to establish critical values. A survey of Ontario laboratories was conducted to determine current practice for critical values in hematology.

Trilineage myelodysplasia and hemophagocytosis associated with systemic lupus erythematosus

Marked trilineage myelodysplasia and hemophagocytosis was noted in the marrow of a 28-year old male of Filipino decent. Six days prior he had been admitted to the hospital for work-up of renal failure, proteinuria, hematuria, and pancytopenia. Past medical history was noteworthy for a 2-year history of a psoriatic like skin rash. The patient also had an emergency room visit for chest pain 1 week prior. He was diagnosed with likely pericarditis and discharged with indomethacin. At admission the patient’s initial laboratory investigation was significant for a creatinine of 270 lmol/L (normal 62–106), potassium of 5.9 mmol/L (normal 3.5–5.1), hemoglobin 104 g/L (normal 130–170), mean corpuscular volume (MCV) of 79.7 fL (normal 80–100), reticulocytopenia of 19.3 3 10/L (normal 25–100), neutrophil count of 2.0 3 10/L (normal 2–7.5), and a platelet count of 105 3 10/L (normal Image 1. Top two rows, left and middle: Evidence of dysplastic erythropoiesis: karyorrhexis (A), cytoplasmic nuclear dysynchrony predominantly in the late normoblasts (B), multinucleation (A,C), and nuclear blebbing (D). 31000 on oil. Top two rows, right: Micromegakaryocyte. 31000 on oil (above). Hemophagocytosis. 31000 on oil (below). Bottom two rows: Normocellular bone marrow with a few strikingly pyknotic megakaryocytes at 3200 and 3400, respectively (A,B), abnormal clustering of megakaryocytes at 3200 (C), mild gelatinous transformation occupying 10% of the core biopsy at3200 (D).

Human T-cell lymphotropic virus type 1-associated adult T-cell leukemia/lymphoma in the Inuit people of Nunavut

Case 1: A 55-year-old Inuit woman from Nunavut presented with a 2-week history of malaise, pain in the right upper abdominal quadrant and mild jaundice. She had a history of tuberculosis, Helicobacter pylori gastritis and peptic ulcer disease. Complete blood count (CBC) results showed a leukocyte