Fatal sickling-associated microvascular occlusive crisis in a young with sickle cell trait

Sickle cell trait (SCT), a heterozygous state characterized by hemoglobin AS, occurs commonly in sub-Saharan Africa, South America, Central America, India, and the Mediterranean countries. SCT is compatible with a normal lifespan and is not commonly regarded as a cause of morbid illness or death compared to its homozygous counterpart. We describe a case of fatal sickling-associated microvascular crisis, identified on post mortem evaluation in a previously undiagnosed 21-year-old military recruit with sickle cell trait. The individual presented with repeated syncope episodes during his training and was autopsied in the pursuit of cardiac anomalies and heat syncope. During the terminal episode, he collapsed and died of severe metabolic complications as he struggled to complete an organized run during routine training activities. To our knowledge, this is the first report of fatal sickling-associated crisis in a military recruit with sickle cell trait from India. This case serves to remind all armed forces and sports physicians of the importance of screening a recruit who is unable to complete exertional physical training for the presence of sickle cell trait.

A 36-year-old man with vomiting, pain abdomen, significant weight loss, hyponatremia, and hypoglycemia.

Diagnosis of Strongyloides stercoralis hyperinfection can be a challenge. The key to a timely diagnosis is to have a high index of suspicion. We present a rare case of a 36-year-old human immunodeficiency virus negative male patient, who was on multidrug therapy for lepromatous leprosy and was treated for type 2 lepra reactions with steroids in the past. The patient presented with vomiting and pain abdomen, persistent hyponatremia, and terminal hypoglycemia. He had features of malnutrition and had a rapid downhill course following admission. A diagnosis of S. stercoralis hyperinfection with sepsis and multiorgan failure, adrenal hemorrhage, and syndrome of inappropriate antidiuretic hormone secretion was established on a postmortem examination.

Double heterozygous hemoglobin Q India/hemoglobin D Punjab hemoglobinopathy: Report of two rare cases.

Cation exchange high performance liquid chromatography (CE HPLC) provides an excellent tool for accurate and reliable diagnosis of various hemoglobin (Hb) disorders. HbQ India is a rare alpha chain variant that usually presents in the heterozygous state. Its presence in double heterozygous state with HbD Punjab is extremely rare. The double heterozygosity for a and b chain variants leads to formation of abnormal heterodimer hybrids, which can lead to diagnostic dilemmas. We report two rare cases of double heterozygous HbQ India/HbD Punjab where the hybrid Hb was seen to elute at retention time similar to HbC on CE HPLC. The first case had unconjugated hyperbilirubinemia at presentation; while, the second case was asymptomatic.

A 60-year-old lady with leonine facies: A rare diagnosis.

Systemic mastocytosis is a rare clonal disorder characterized by mast cell infiltration of one or more organs, with or without skin involvement. Leonine facies is a rare presentation and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face as a result of long standing disease. Bone marrow aspiration and biopsy findings in a 60-year-old woman, who had extensive cutaneous infiltration due to systemic mastocytosis, resulting in 'leonine facies,' are described, and causes of leonine facies are discussed.

Pseudotumoral hepatic tuberculosis with pericardial abscess.

We report the postmortem findings in a 28-year-old immunocompetent male patient, a rare case of tuberculous liver abscesses with concomitant pericardial abscess in the absence of pleuropulmonary or splenic involvement, who continued to be a diagnostic dilemma. This case report illustrates the difficulty in reaching the correct diagnosis in case of hepatic masses, which are most often confused with carcinoma of the liver, primary or metastatic and, hence, have been aptly referred to as pseudotumoral hepatic tuberculosis in the past.

Disseminated acanthamoeba infection in a renal transplant recipient: a case report.

Acanthamoeba species are free-living amoebae that are the causative agents of chronic granulomatous meningoencephalitis, amoebic keratitis, pulmonary lesions, cutaneous lesions and sinusitis. Immunocompromised individuals are particularly susceptible to infections with Acanthamoeba, which can be disseminated at times. We herewith report the autopsy findings of disseminated Acanthamoeba infection in a 36-year-old female, a renal transplant recipient on immunosupressants for last four years. Central nervous system showed Acanthamoeba associated chronic granulomatous meningoencephalitis, with predominant perivascular infiltrate of amoebic cysts, trophozoites and inflammatory cells. Both lungs and pancreas also showed infiltration with Acanthamoeba.