[Hemophagocytic lymphohistiocytosis as a manifestation of visceral leishmaniasis]. / Hemofagocytující lymfohistiocytóza jako manifestace viscerální leishmaniózy.

A 7-year-old previously healthy Czech boy was admitted due to fever, hepatosplenomegaly and pancytopenia. Aspiration of bone marrow revealed no signs of hemoblastosis (nor hemophagocytosis). He was treated with antibiotics and virostatics without effect. Progression of hepatosplenomegaly and pancytopenia induced suspicion of hemophagocytic lymphohistiocytosis (HLH). Five weeks later, bone marrow hemophagocytosis of erythrocytes, nuclear elements and platelets was detected. He was given corticoids and intravenous immunoglobulins and transferred to our haematology department. Laboratory findings of mild pancytopenia, hypofibrinogenaemia, hyperlipidaemia and elevated levels of ferritin, LDH and immunoglobulins were compatible to the diagnosis of HLH. Immunologic evaluation revealed T-lymphocyte activation. Appropriate immunosuppressive treatment with Dexamethasone, etoposide and Cyclosporine A was launched, followed by transient subside of fever and improvement of peripheral blood count, but not regression of hepatosplenomegaly. Four weeks later, relapse of fever and deterioration of blood count led to intensification of immunosuppression. However, no effect was evident. Moreover, hypertrophic cardiomyopathy with ventricular arrhythmia occurred. Treatment with antilymphocytic globulin for resistant course of HLH was planned. Before that, a fifth bone marrow aspiration was performed. Surprisingly, many Leishmania amastigotes were observed within marrow macrophages. Leishmania infection was confirmed by positive serology. Immunosuppressive treatment was withdrawn and changed for causal treatment with liposomal Amphotericin B. Positive clinical effect with subside of fever was evident in ten days, splenomegaly gradually resolved during three weeks, restoration of normal blood count lasted six weeks. No relapses of HLH nor leishmaniasis occurred. In control bone marrow aspirate performed three months later, the parasites were not detected. Ten months after the event, the patient is in complete remission of HLH with normal immunologic parameters. Most probably, he contracted visceral leishmaniasis during a visit of a Neapol area in Italy 3 months before the onset of the disease.

The review of imported visceral leishmaniosis in the Czech Republic.

BACKGROUND: From the late 1950s through 2000, a total of 8 cases of imported visceral leishmaniosis (VL) were registered in the Czech republic. OBJECTIVES: The authors were made to point to the issue of imported VL by the fact 3 cases of this disease (imported from East Africa, Croatia, and southern Italy) were reported in 1999, plus another one (again imported from Croatia) in 2000. METHODS: The case reports of 4 cases of imported VL are presented. They are cases 5-8 ever reported in the Czech Republic. RESULTS: The infection manifested itself by fever, marked splenomegaly, leukopenia, thrombocytopenia, and rapid weight loss. The diagnosis was confirmed by the microscopic finding of amastigotes in punctate obtained from bone marrow, liver, spleen and, also, by serology. All the patients were successfully treated with amphotericin B. CONCLUSION: Infection by VL should be considered when establishing the diagnosis not only in patient returning from endemic regions and show hepatosplenomegaly, fever, leukopenia, and thrombocytopenia. Given the long incubation time, VL may be encountered also in foreigners who had lived in the above regions. Besides, the diagnosis of VL should also be considered in immunocompromised individuals. (Ref. 27.).

Pathogenesis of pulmonary hypertension in tuberculosis.

The pathogenic factors involved in the increase of pressure in the pulmonary artery in 55 tuberculous patients with manifest, and 50 patients with latent pulmonary hypertension were examinated and analyzed in detail. The authors consider decisive the restriction of pulmonary blood bed together with the decrease of functional parenchyma. In the increase of arterial pressure also the functional vasoconstriction participates. An important role plays here hypoxemia adversely affecting the cardiac function. Hypoxemia, of all pathogenic factors, represents the most serious limitation of pulmonary diffusion, though other agents may not be primarily important for the onset and development of pulmonary hypertension. The originating pulmonary hypertension in tuberculosis does not necessitate compensating mechanisms such as polyglobulia, hypervolemia, or increase of the minute cardiac volume normally present in severe and protracted hypoxemia. The prognosis of patients with the mean pulmonary artery pressure higher than 30 mmHg (3.33 kPa) is very bad, the median survival being about 10 months. Contrarily, a low-grade pulmonary hypertension may not, per se, provide sinister prognostic outlooks. Here rather some other agents capable of influencing the perspectives seen to be of major importance.