Hemophagoctic lymphohistiocytosis--recent concept.

Hemophagocytic lymphohistiocytosis is a rare condition characterized by highly stimulated but inactive immune response. The disease may be inherited or acquired due to infections, collagen vascular diseases and malignancies. The pathological hallmark of the syndrome is aggressive proliferation of macrophages and histiocytes. Decreased NK cell activity results in increased T cell activation resulting production of large quantities of interferon gamma (IFN gamma), tumor necrosis factor alpha (TNF alpha) and granulocyte macrophage colony stimulating factor (GM-CSF). This causes sustained macrophage activation and tissue infiltration as well as production of interleukin 1 (IL1) and interleukin 6 (IL6).The resulting inflammatory reaction causes extensive damage and associated symptoms. Patients with HLH commonly present with high fever, anemia and splenomegaly. Minimal diagnostic parameters are a complete hemogram, liver function test, serum triglycerides and ferritin, coagulation profile including fibrinogen and bone marrow aspiration. Two highly sensitive diagnostic marker are an increased plasma concentration of the alpha chain of soluble IL2 receptor (CD25) and impaired NK cell activity. Hyperinflammation can be treated with steroid, Cyclosporine prevents T lymphocytes and immunoglobulin infusion helps to control the infection. Etoposide may be life saving specially in case of HLH with Ebstein Barr Viruses infection. The Histiocyte Society in 1994 developed a common treatment protocol (HLH-94). In January 2004 a revised HLH treatment protocol was opened entitled HLH-2004, which is based on HLH-94 with minor modifications. There is a high remission rate on the HLH-94 and HLH-2004 treatment protocols.

Transverse testicular ectopia with persistent Muellerian duct syndrome.

A one-year-old boy was admitted with complaints of swelling in the left inguinoscrotal region and an empty right scrotum since birth. The inguinoscrotum contained two ovoid solid swellings one above the other. The swellings were testes like in feel, size and shape. The diagnosis was transverse testicular ectopia. The case was managed by surgery. Tissue from gonads, the tubular structures and the fallopian tubes were sent for histopathological examination for confirmation. This was a case of an otherwise normal male with transverse testicular ectopia with persistent Muellerian duct syndrome in the left inguinoscrotal hernial sac.

Epidemiology of meningococcal carrier state amongst recruits of a military training centre.

An epidemiological study was carried out to determine meningococcal carrier state amongst recruits of a military training centre. 360 recruits with divergent socioeconomic, ethnic backgrounds were studied. Epidemiological factors such as age, religion, educational status, housing conditions, family structure were considered. The overall carrier rate was 11.94%. Carrier rate was higher amongst recruits from poor background and joint families. No association was found between carrier state and smoking as well as those suffering from upper respiratory tract infection. Carrier rate was aslo studied in relation to age, marital status, educational back ground and region.

Bilateral pulmonary aneurysm in Behçet's disease (a case report).

While investigating a chest opacity in Behcet's disease possibility of pulmonary aneurysm should be considered. Contrast enhanced CT is a safe diagnostic method which is useful in confirming or excluding the presence of an aneurysm in such cases. We report here the first case of bilateral pulmonary aneurysms in Behcet's disease.

Hamartoma--a benign intraluminal tumor of the oesophagus (a case report).

Benign tumours of the oesophagus are rare, representing less than 1% of all oesophageal neoplasms. Most of them are intramural leiomyomas while the other benign tumours are encountered only infrequently; among these pedunculated intraluminal hamartomas form a particularly rare group. We present here one such case.

Pulmonary pseudoaneurysm.

A case of pseudoaneurysm with erosion of both pulmonary and bronchial vessels is reported. The diagnosis was confirmed by computed tomography followed by intravenous digital subtraction angiography. It was removed surgically.

Adreno cortical carcinoma in children--a case report of three cases.

Three cases of adrenal carcinomas are reported with emphasis on different radiological imaging techniques. It is felt that sonography and C.T. both have an important role in diagnosing masses of suprarenal region.

Primary mediastinal embryonal cell carcinoma.

It is now widely accepted that germinal cell tumors can arise primarily in the mediastinum. Primary extragenital seminoma has been documented arising in the mediastinum but very few reports are available about primary mediastinal embryonal cell carcinoma. We report a case of this rare condition with radiological and CT features.

Calcified hypernephroma. A case report.

Pattern of classification in an renal neoplasm is variable. If calcium is located nonperipherally (within the mass) it is more likely to be a renal cell carcinoma. But peripheral calcification does not rule out malignancy.