Infection-associated haemophagocytosis: the tropical spectrum.

Reactive proliferation and inappropriate activation of mature histiocytes with haemophagocytosis (HP) may occur in association with a wide variety of infections, neoplasms, collagen vascular diseases, and acquired and inherited immunodeficiency states. The association with infections is particularly important because overwhelming HP can obscure the typical clinical features of the primary disease and negatively affects outcome. A high index of suspicion is required for early recognition of associated HP as the cause of cytopenias. Institution of specific therapy is crucial for survival. This study highlights the wide spectrum of tropical diseases that can have associated reactive HP. Thirty cases with documented prominent HP on bone marrow aspiration smears were reviewed. Twenty-one (69%) of the marrows were from patients who had common tropical infections: malaria, typhoid and visceral leishmaniasis and 11 of 15 patients (73%) who were followed up improved on specific infection-directed and supportive measures. The presence of severe HP in bone marrow smears correlated with marked cytopenias. Recognition of HP in this geographical region should stimulate the search for one of these infections as early institution of specific therapy is crucial for patient survival.

Isolated cardiac aspergillosis.

A 40-year-old man, a known case of Wolff-Parkinson-White syndrome, was admitted to the hospital in an unconscious state. In spite of medical treatment, the patient died within two hours of admission. At autopsy, the deceased was found to have aspergillosis involving the interatrial septum, aortic valve and root of the aorta. The rest of the organs were unremarkable. The patient did not show any obvious signs of being immunocompromised. We report this case of isolated cardiac aspergillosis in an apparently healthy individual.

Disseminated histoplasmosis with reactive hemophagocytosis: aspiration cytology findings in two cases.

Two cases of disseminated histoplasmosis associated with reactive hemophagocytic syndrome are described. The clinical presentation was with prolonged unexplained fever and hepatosplenomegaly. On a strong clinical possibility of tuberculosis, antitubercular treatment was initiated in both patients. Lymph node (case 1), splenic (case 2), and bone marrow aspiration, however, showed sheets of proliferating histiocytes, and intracellular and extracellular histoplasma organisms. Aspiration cytology was thus valuable in establishing the final diagnosis. The patients had a fulminant clinical course and died of hemorrhagic shock within 48 hr of hospital admission before specific therapy could be initiated. Histoplasmosis can mimic tuberculosis clinically. There is a need for an increased awareness of the clinicopathological spectrum of histoplasmosis, especially its rarer manifestations as hemophagocytic syndrome. In suspected cases, aspirations from the lymph node, liver, and spleen can be performed safely and should be utilized for early diagnosis.

Haemostatic changes in children with cyanotic and acyanotic congenital heart disease.

This study was undertaken to screen children with congenital heart disease for coagulation abnormalities and to compare the groups of cyanotic and acyanotic children with congenital heart disease with respect to abnormalities of the coagulation system. Following investigations were done in all the patients: complete blood count, erythrocyte sedimentation rate, peripheral smear examination, bleeding time, prothrombin time, activated partial thromboplastin time, assay of fibrinogen, D-dimer, factors VII and VIII and antithrombin III. Red cell indices were determined in 12 control, 12 acyanotic and 20 cyanotic children. Twenty-five patients each, with echocardiographically proven cyanotic and acyanotic congenital heart disease under 12 years of age constituted the study group; as many children of the same age group were included as the control group. The results showed isolated abnormalities of laboratory tests with equal frequency (28%) in acyanotic and cyanotic groups but coexisting abnormalities of more than one test were seen in significantly larger number of cyanotic children (5/25 and 16/25, respectively). A significant association was noted between thrombocytopenia and a high haematocrit in cyanotic patients. It is concluded that laboratory abnormalities of tests of haemostasis are more common in cyanotic congenital heart disease patients. The patterns of laboratory abnormalities suggest a chronic compensated disseminated intravascular coagulation at a subclinical level, reduced synthesis of clotting factors and/or deranged platelet aggregation in different subgroups of patients.

Medullary thyroid carcinoma with melanin production--a case report.

Melanin production in medullary thyroid carcinomas is rare. The present case illustrates melanin and other atypical features of medullary carcinoma of the thyroid in a fifty year old female. The diagnosis was suggested on the cytomorphological features seen on fine needle aspiration cytology smears. On histo-pathological examination the tumor was extensively pigmented with frequent mitosis. Amyloid was conspicuously scarce. Confirmation of diagnosis was done by immunohistochemical positivity for calcitonin and HMB-45 on tissue sections. The case is being presented in view of its rarity and distinct immunoreactivity. Review of literature is done and the implications of such dual positivity in the histogenesis and divergent phenotype of this tumor are discussed.

Renal dysplasia: an autopsy study of associated congenital malformations.

A retrospective analysis of infant autopsies in the last 14 years was done with the aim of determining the frequency of associated malformations. Renal dysplasia comprised 36 of 594 autopsies done and was the commonest congenital urologic malformation (6.06% of all infant autopsies). Histologically all cases were characterised by hallmarks of disordered metanephric differentiation and organisation. Extrarenal malformations were seen in all (100%) of the cases of which other urologic anomalies were the commonest (28) followed by those of musculoskeletal (18), gastrointestinal (13), cardiovascular (10) and central nervous system (6). Syndromic association was seen in 5 (14%) cases. There were twenty two percent still births and 47% infants died perinatally. A thorough search for systemic malformations is mandatory in all cases of renal dysplasia as these may influence the survival significantly. Cystic renal dysplasia should be differentiated from other causes of cystic renal diseases.

Sertoli Leydig cell tumor with malignant heterologous elements and raised alpha-fetoprotein: a case report.

A case of a Sertoli Leydig cell tumor in a young female with virilizing symptoms and an androgenic endocrine profile with raised serum alpha-fetoprotein is presented. The tumor consisted predominantly of malignant epithelial and mesenchymal heterologous elements. Such a combination in the same tumor has to the best of our knowledge not been previously reported in English literature. The Leydig cells were immunohistochemically positive for alpha-fetoprotein. Sertoli Leydig cell tumors should be included in the differential diagnosis of tumors with raised alpha-fetoprotein. Chemotherapy in addition to surgery has been recommended for these tumors.

Basaloid-squamous carcinoma. A distinct histopathological entity.

Basaloid Squamous Carcinoma (BSC) is an uncommon aggressive neoplasm occurring in the upper aerodigestive tract. Lung as a primary site has only recently been reported. A combination of microscopic features is required to make a diagnosis and to differentiate it from other more common malignancies in these sites. Three cases of BSC including one in the lung are reported. There are no prior published reports of this neoplasms in Indian literature. The study highlights the typical microscopic features and the diagnostic difficulties which may be encountered.