[Visceral Leishmaniasis in an Immunocompetent Patient: A Case Report]. / Leishmaniose Visceral em Doente Imunocompetente: Relato de um Caso.

Leishmaniasis is a parasitic disease transmitted by the bite of female sandflies that occurs in tropical and subtropical climate regions. Visceral leishmaniasis is the most serious manifestation of the disease, leading to a 95% mortality rate after two years of infection if untreated. Visceral leishmaniasis is frequently associated with immunocompromised states, with the human immunodeficiency virus being the most prevalent. Most cases of visceral leishmaniasis are caused by the species Leishmania donovani and Leishmania infantum, the latter being the endemic species in the Mediterranean basin. In Portugal, the number of reported cases of visceral leishmaniasis has decreased in the last few years, with 15 cases reported between 2017 and 2021. The authors present a case of visceral leishmaniasis in an immunocompetent patient who manifested the classic pentad: fever, weight loss, hepatosplenomegaly, pancytopenia and hypergammaglobulinemia. The diagnosis was made by the observation of amastigotes of the Leishmania infantum species in the bone marrow aspirate examination, and the patient was successfully treated with liposomal amphotericin B.

A leishmaniose é uma doença parasitária transmitida através da picada de flebotomíneos fêmea e que ocorre em regiões de clima tropical e subtropical. A leishmaniose visceral é a forma mais grave da doença, com uma mortalidade de 95% aos dois anos de infeção, quando não tratada. A leishmaniose visceral associa-se frequentemente a estados de imunossupressão, sendo a coinfeção com o vírus da imunodeficiência humana o mais prevalente. A maioria dos casos de leishmaniose visceral é causada pelas espécies Leishmania donovani e Leishmania infantum, sendo esta última a espécie endémica na bacia do Mediterrâneo. Em Portugal, o número de casos reportados de leishmaniose visceral tem vindo a diminuir nos últimos anos, sendo que entre 2017 e 2021 foram reportados 15 casos. Os autores apresentam um caso de leishmaniose visceral numa doente imunocompetente, que manifestou a pêntade clássica: febre, perda ponderal, hepatoesplenomegalia, pancitopenia e hipergamaglobulinemia. O diagnóstico foi feito pela observação de amastigotas da espécie Leishmania infantum no exame anatomopatológico da medula óssea e a doente foi tratada com sucesso com anfotericina B lipossómica.

Bone Marrow Langerhans Cell Histiocytosis in Association with Kasabach-Merritt Syndrome: The Difficulty of a Differential Diagnosis.

Langerhans cell histiocytosis is a rare haematological disorder with variable clinical findings and a high mortality rate. On the other hand, Kasabach-Merritt syndrome is of rare onset at adult age, requiring the simultaneous presentation of vascular lesion, thrombocytopenia, and consumptive coagulopathy. We present the first reported case of both diseases in a single patient and highlight the difficulties of diagnostic. A 69-year-old woman with immune thrombocytopenic purpura underwent surgery for the removal of giant skin haemangiomas. During post-operative care, intravascular disseminated coagulopathy developed. After weeks of corticosteroids and immunosuppressive therapy with no clinical improvement, pulmonary tuberculosis was diagnosed and appropriate treatment initiated. Despite all the efforts, the patient's clinical condition kept worsening and she eventually died. An autopsy revealed bone marrow Langerhans cell histiocytosis. In this case, the patient's autoimmune background together with tuberculosis and intravascular disseminated coagulopathy masked the presentation and made the diagnosis of a rapidly progressive fatal disease very difficult.

Prospective Comparison of Transient Elastography Using Two Different Devices: Performance of FibroScan and FibroTouch.

PURPOSE: Transient elastography (TE) using FibroScan (FS) has been established to non-invasively assess liver fibrosis and steatosis. The aim of this study was to compare the recently introduced FibroTouch (FT) device with the established FS with respect to liver stiffness and CAP. PATIENTS AND METHODS: Thirty-nine patients with and without liver disease were included. All patients were measured three times with FS (FibroScan 530 compact, Echosens, France) and FT (FibroTouch-FT100, Wuxi Hisky Med, China). For FS, M and XL probe were used according to the manufacturer's specifications. For steatosis, CAP and the comparable FT equivalent UAP (ultrasound attenuation parameter) was determined. Finally, FT and FS were explored in liver tissue-mimicking phantoms. RESULTS: LS between FS and FT correlated well with r=0.91. Root-mean-square (RMS) of the coefficient of variation for LS was better in FS (11.1% vs 27.4%). Bland-Altman analysis showed a 3.1 kPa mean overestimation of LS by FT. In addition, UAP strongly and linearly depended on the BMI following UAP=3.02 × BMI+186. In phantoms, a similar relation was found with UAP (phantom)= 3.78 × BMI + 146 suggesting that UAP is directly calculated from entered BMI instead of assessing shear-wave attenuation. Consequently, RMS-CV was lower for FT (6.0% vs 9.7%). However, if using different BMI, CV-RMS for FT increased to 12.7%. LS of a patient with manifest liver cirrhosis and ascites was 38.8 kPa using the FS-XL probe but almost normal with FT (7.2 kPa). CONCLUSION: Although LS by FT shows good correlation with LS-FS, it has larger variation, continuously overestimates LS and completely fails in ascites. Moreover, FT-UAP seems to be a misleading parameter for steatosis assessment because it is at least in part calculated from mandatory entered patient data. In conclusion, novel LS cut-off values need to be defined for LS-FT and usage of UAP is not recommended.