CD20 positive T-cell lymphoma/leukemia: a rare entity with potential diagnostic pitfalls.

Mature T-cell neoplasms are relatively uncommon, accounting for approximately 10% of all non-Hodgkin lymphomas. This category of hematopoietic neoplasms is clinically aggressive and shows a poor response to therapy and shortened survival. The antigen CD20 has long been thought to be a specific marker for B-cell lineage and has been used to help differentiate T-cell and B-cell neoplasms. We present two cases of a rare subset of T-cell leukemia/lymphoma having a unique immunophenotype, both being CD20+. The significance of CD20 antigen in T-cell lymphomas is yet to be determined, but may allow treatment with novel therapeutic agents (eg, rituximab, a recombinant anti-CD20 monoclonal antibody).

Use of recombinant activated factor VII concentrate to control postoperative hemorrhage in complex cardiovascular surgery.

BACKGROUND: Complex cardiovascular surgery often results in postoperative hemorrhage. Excessive blood product use may cause systemic thrombosis, end-organ dysfunction, and edema preventing chest closure. Recombinant activated factor VII (rFVIIa) concentrate may decrease hemorrhage where other treatment measures failed. We reviewed our experience with rFVIIa after complex cardiovascular surgery. METHODS: A retrospective review evaluating 846 complex cardiovascular surgery patients of whom 36 received rFVIIa between January 1, 2001, and December 31, 2006, was performed. Efficacy and safety data were collected for the entire cohort in addition to delayed sternal closure requirements, reoperation, and operative mortality in the patient cohort temporally separated into two groups (pre-rFVIIa era, 2001 to 2003, 1 patient received rFVIIa; rFVIIa era, 2004 to 2006, 35 patients received rFVIIa). RESULTS: A total of 36 patients received 41 rFVIIa doses with an in-hospital survival of 91.7%. Hemorrhage was controlled in 83.3% of patients, with 1 dose sufficient in 75.0%. There was a significant decrease (p < 0.005) in all blood product requirements post-rFVIIa compared with pre-rFVIIa administration. In the intensive care unit (n = 6), rFVIIa significantly reduced chest tube output (p = 0.028) and prevented reexploration for bleeding in 5 patients. The requirement for delayed sternal closure was significantly higher in the pre-rFVIIa era versus the rFVIIa era (p = 0.011). The incidence of thrombosis in all patients receiving rFVIIa was 11.1%. In the rFVIIa era, a higher incidence of postoperative renal failure (p = 0.005) and pneumonia (p < 0.002) was detected in patients receiving rFVIIa. CONCLUSIONS: Recombinant activated factor VII appears to be effective in patients with refractory coagulopathy undergoing high-risk cardiovascular surgery.

Natural death in the forensic setting: a study and approach to the autopsy.

Although unexpected natural deaths comprise a large proportion of the cases seen in the forensic setting, the investigation of these cases remains a unique challenge. Some cases are straightforward; however, others require special examination and dissections, as well as the use of special dissections and ancillary studies. For the forensic investigator to accurately assign a cause and manner of death, one must be aware of the differential diagnoses, common scenarios, and typical victim. The authors reviewed all forensic cases referred to the Forensic Section of the Medical University of South Carolina over a 15-year period from January 1989-December 2003. The number of natural deaths occurring in adults (age 18 years and older) was 6932, compared with the overall number of 13,227 cases. The natural deaths were then analyzed as to autopsy findings, cause of death, cardiac versus noncardiac, age, race, and gender. Current techniques, special dissections, and ancillary studies that can assist in the classification of these deaths in the forensic setting are reviewed.

Pregnancy-associated deaths: a 15-year retrospective study and overall review of maternal pathophysiology.

Pregnancy-related death is defined by the International Classification of Diseases, Tenth Revision (ICD-10) as the death of a woman while pregnant or within 42 days of termination of pregnancy, irrespective of the cause of death. In the year 2000, a collaborative effort involving World Health Organization (WHO), UNICEF, and UNFPA estimated 660 maternal deaths in the United States. This averages 11 maternal deaths per 100,000 live births reported. Many pregnancy-associated deaths are not easily identified as such since the presence of a recent or current pregnancy may not be listed on the death certificate. Thus, the WHO estimates that in the United States, the maternal mortality is approximately 17/100,000 pregnancies. This is significantly higher than the goal set by the US Department of Health and Human Services in Healthy People 2010, which sets the target for maternal mortality at less than 3.3/100,000 live births. The most common causes of maternal death vary somewhat from region to region in the United States. They include pulmonary thromboembolism, amniotic fluid embolism, primary postpartum uterine hemorrhage, infection, and complications of hypertension including preeclampsia and eclampsia. Pulmonary disease, complications of anesthesia, and cardiomyopathy also are significant contributors to maternal mortality in some populations. The death of a pregnant or recently pregnant individual poses a wide scope of challenges to the forensic pathologist and investigator. The pathologist must have a broad knowledge of the physiologic and biochemical changes that occur during pregnancy, as well as the clinical and pathological manifestation of these changes. Conditions that may be "benign" in the nonpregnant individual may be lethal in the puerperal period. In addition, it should be kept in mind that deaths during pregnancy may be due to unnatural causes. Accident, homicide, and suicide must be ruled out in each case. The authors reviewed all forensic cases referred for autopsy to the Forensic Section of the Medical University of South Carolina from January 1989 through December 2003. All decedents listed as pregnant or postpartum were analyzed as to maternal age, race, past medical history, previous pregnancies and outcome, prenatal care, gestational age, fetal or neonatal outcome, location of delivery, placental findings, maternal autopsy findings, toxicology, cause of death, manner of death, and fetal or neonatal autopsy findings. The authors present this retrospective study to better determine the factors leading to maternal demise and discuss the autopsy/ancillary techniques useful in determining the cause of death in this challenging area.

Mosaic duplication 1(q11q44) in an infant with nephroblastomatosis and mineralization of extraplacental membranes.

Partial trisomy of 1q is rare. Only 32 cases of isolated partial trisomy 1q have been previously reported. From these cases, a characteristic phenotype is beginning to emerge. We present a case of mosaic duplication of 1q [46,XX,dup (1)(q11q44)/46,XX]. Many features of our patient have been described in previous patients, thus supporting the emerging phenotype. Two particular features, however, have not been previously described. The present case demonstrated extensive mineralization of the extraplacental membranes and bilateral nephromegaly, with an extreme form of diffusely hyperplastic perilobar nephroblastomatosis. Clinical comparison is made between our case and previously reported cases, and the clinical significance of the unique findings are reviewed and discussed.

Pathologic findings in malignant hyperthermia: a case report and review of literature.

Malignant hyperthermia (MH) is a rare, potentially lethal disorder of skeletal muscle calcium homeostasis characterized by muscle contracture and life-threatening hypermetabolic crisis following exposure to halogenated anesthetics and depolarizing muscle relaxants. Susceptibility to MH results from mutations in calcium channel proteins that mediate excitation-contraction coupling, with the ryanodine receptor calcium release channel (RyR1) representing the major locus. The mode of inheritance appears to be autosomal dominant with variable penetrance. The authors report the death of a 60-year-old white male with a history of low back pain. He had undergone 2 back surgeries previously, the first occurring 10 years prior to his current presentation. Both previous procedures were done under generalized anesthetic with no complications. Recently, he developed stenosis and presented for fusion of vertebrae L3 and L4. The procedure was performed under general anesthetic including sevoflurane, with no intraoperative complications. The anesthesiologist noted that, near the end of the 2-hour procedure, the decedent's CO2 levels were slightly elevated. After the procedure, the decedent was extubated, the temperature probe which had been recording normal values was removed, and he was rolled from ventral to dorsal position. He immediately became hypotensive and bradycardic. Lifesaving interventions were begun. Subsequently, he went into cardiac arrest, at which time the temperature probe was reinserted into the trachea, where it read a body temperature of 109 degrees F. Malignant hyperthermia protocol was initiated, and interventions continued for over 2 hours, at which time they failed. At autopsy, the abdomen contained 1800 mL of blood, and bilateral hematomas were present in the psoas muscles. The authors present this case of clinically apparent malignant hyperthermia, discuss how to approach such a case, the gross and microscopic findings, ancillary studies, and a review of the literature.