Rare association of consumptive coagulopathy in visceral leishmaniasis: A case report.

Visceral leishmaniasis is a tropical parasitic disease caused by the species of the genus Leishmania infantum. The clinical picture includes fever, splenomegaly, leucopenia, anaemia and hypergammaglobulinaemia. There may also be a drop in plasma fibrinogen levels or an increase in plasma fibrinolytic activity. Furthermore, visceral leishmaniasis may be the trigger for secondary haemophagocytic lymphohistiocytosis. On the other hand, disseminated intravascular coagulation may also result. The International Society of Thrombosis and Hemostasis has recommended the use of a scoring system for disseminated intravascular coagulation. An association between visceral leishmaniasis and consumption coagulopathy is not frequent. Our systematic literature review from 1967 to 2019 pointed to the report of only 16 cases. Our case demonstrates that it is necessary to be aware of the existence of this association.

Interleukin-6 in pregnancy with sickle cell disease

ABSTRACT Background: Despite advances in health care for sickle cell disease patients, as well as in the improvement in reproductive issues mainly in women with the disease, pregnancy is still a challenge, both for the mother and the child, with high rates of maternal and fetal morbidity and mortality. Besides their chronic hemolytic status and vaso-occlusive events that confer systemic complications, pregnant women also have higher rates of pain episodes, infections, abortion, intrauterine growth retardation, pre-term births, eclampsia, stillbirth and the hemolysis, elevated liver enzymes and low platelets syndrome. The physiologic mechanisms of the disease in pregnancy are still unknown and chronic inflammatory responses may interfere in the adverse outcomes. The cytokine and chemokine profiles in pregnancy with sickle cell disease remain unknown. The aim of this study was to evaluate the cytokine profile of the inflammatory response of pregnant women with sickle cell disease. Method: Blood samples from 20 pregnant women with sickle cell disease, 24 women with sickle cell disease in steady state, 16 healthy pregnant women and a control group with 9 women at childbearing age were assayed for interleukin-6. Main results: Pregnant women with sickle cell disease presented high serum levels of interleukin-6, compared to healthy pregnant women (p = 0.0115). Conclusion: These data suggest that the increased production of interleukin-6 may occur during pregnancy with sickle cell disease and that the role of this cytokine in the sickle cell disease pathophysiology and pregnancy complications should be further studied.

Interleukin-6 in pregnancy with sickle cell disease.

BACKGROUND: Despite advances in health care for sickle cell disease patients, as well as in the improvement in reproductive issues mainly in women with the disease, pregnancy is still a challenge, both for the mother and the child, with high rates of maternal and fetal morbidity and mortality. Besides their chronic hemolytic status and vaso-occlusive events that confer systemic complications, pregnant women also have higher rates of pain episodes, infections, abortion, intrauterine growth retardation, pre-term births, eclampsia, stillbirth and the hemolysis, elevated liver enzymes and low platelets syndrome. The physiologic mechanisms of the disease in pregnancy are still unknown and chronic inflammatory responses may interfere in the adverse outcomes. The cytokine and chemokine profiles in pregnancy with sickle cell disease remain unknown. The aim of this study was to evaluate the cytokine profile of the inflammatory response of pregnant women with sickle cell disease. METHOD: Blood samples from 20 pregnant women with sickle cell disease, 24 women with sickle cell disease in steady state, 16 healthy pregnant women and a control group with 9 women at childbearing age were assayed for interleukin-6. MAIN RESULTS: Pregnant women with sickle cell disease presented high serum levels of interleukin-6, compared to healthy pregnant women (p=0.0115). CONCLUSION: These data suggest that the increased production of interleukin-6 may occur during pregnancy with sickle cell disease and that the role of this cytokine in the sickle cell disease pathophysiology and pregnancy complications should be further studied.

The first reported case of factor V Leiden mutation with agenesis of superior vena cava: A case report.

RATIONALE: Total absence of superior vena cava (ASVC) is a very rare anomaly, and the patient usually suffers from superior vena cava syndrome (SVCS) or conduction disturbances. PATIENT CONCERNS: We report a 36-year-old white male, born and living in Brazil, without comorbidities presented to hematologist thrombotic episodes even under anticoagulant therapy. On his first hematologic appointment, he had no active complaints except by the fullness after meals, and his physical examination presented remarkable collateral circulation in the chest. DIAGNOSES: Congenital ASVC associated with factor V Leiden mutation. OUTCOMES: In his magnetic resonance angiography of the thorax, a great amount of collateral circulation and communication of the azygos and hemiazygos veins with inferior vena cava were evident, as well as the absence of the upper cava vein. Furthermore, heterozygous genetic mutation was found for Leiden factor V. LESSONS: This case gives us the lesson that we need to include ASVC in the differential diagnosis of SVCS. The importance of the V-Leiden factor as a joint risk with this congenital defect for venous thromboembolism episodes was also highlighted.

Profile of Reproductive Issues Associated with Different Sickle Cell Disease Genotypes / Perfil reprodutivo associado aos diferentes genótipos da doença falciforme

Abstract Purpose To describe the reproductive variables associated with different sickle cell disease (SCD) genotypes and the influence of contraceptive methods on acute painful episodes among the women with the homozygous hemoglobin S (HbSS) genotype. Methods A cross-sectional study was conducted between September of 2015 and April of 2016 on 158 women afflicted with SCD admitted to a hematology center in the Northeast of Brazil. The reproduction-associated variables of different SCD genotypes were assessed using the analysis of variance (ANOVA) test to compare means, and the Kruskal-Wallis test to compare medians. The association between the contraceptive method and the acute painful episodes was evaluated by the Chi-square test. Results Themean age of women with SCD was 28.3 years and 86.6% were mixed or of African-American ethnicity. With respect to the genotypes, 134 women (84.8%) had HbSS genotype, 12 women (7.6%) had hemoglobin SC (HbSC) disease genotype, and 12 (7.6%) were identified with hemoglobinopathy S-beta (S-β) thalassemia. The mean age of HbSS diagnosis was lower than that of HbSC disease, the less severe formof SCD (p < 0.001). The mean age ofmenarche was 14.8 ± 1.8 years for HbSS and 12.7 ± 1.5 years for HbSC (p < 0.001). Among women with HbSS who used progestin-only contraception, 16.6% had more than 4 acute painful episodes per year. There was no statistically significant difference when compared with other contraceptive methods. Conclusion With respect to reproduction-associated variables, only the age of the menarche showed delay in HbSS when compared with HbSC. The contraceptive method used was not associated with the frequency of acute painful episodes among the HbSS women.

Resumo Objetivo Descrever as variáveis reprodutivas em diferentes genótipos da doença falciforme (DF) e a influência dos métodos contraceptivos na frequência das crises álgicas em mulheres com homozigose da hemoglobina S (HbSS). Métodos Estudo de corte transversal realizado entre setembro de 2015 e abril de 2016 com 158 mulheres com DF atendidas em um centro de hematologia no Nordeste do Brasil. As variáveis reprodutivas dos diferentes genótipos da DF foram avaliadas utilizando-se o teste de análise de variância (ANOVA) para comparação de médias e o teste de Kruskal-Wallis para comparação de medianas. A associação entre o método contraceptivo e a frequência das crises álgicas foi avaliada pelo teste Qui-quadrado. Resultados A idade média das mulheres com DF foi de 28,3 anos e 86,6% eram afrodescentes. Em relação aos genótipos, 134 mulheres (84,8%) tinham genótipo HbSS, 12 mulheres (7,6%) tinham genótipo para doença da hemoglobina SC (HbSC) e 12 (7,6%) foram identificadas com beta talassemia (S-β). A idade média do diagnóstico de HbSS foi menor do que a da HbSC, sendo esta a forma menos grave da DF (p < 0,001). A idade média da menarca foi de 14,8 ± 1,8 anos para HbSS e de 12,7 ± 1,5 anos para HbSC (p < 0,001). Entre as mulheres com HbSS que fizeram contracepção com progesterona isolada, 16,6% apresentaram mais de 4 episódios de crises álgicas agudas por ano. Não houve diferença estatisticamente significativa quando comparado com outros métodos anticoncepcionais. Conclusão Em relação às variáveis reprodutivas, apenas a idade da menarca apresentou atraso no HbSS em relação ao HbSC. O método anticoncepcional utilizado não foi associado à frequência de crises álgicas entre as mulheres com HbSS.

Profile of Reproductive Issues Associated with Different Sickle Cell Disease Genotypes.

Purpose To describe the reproductive variables associated with different sickle cell disease (SCD) genotypes and the influence of contraceptive methods on acute painful episodes among the women with the homozygous hemoglobin S (HbSS) genotype. Methods A cross-sectional study was conducted between September of 2015 and April of 2016 on 158 women afflicted with SCD admitted to a hematology center in the Northeast of Brazil. The reproduction-associated variables of different SCD genotypes were assessed using the analysis of variance (ANOVA) test to compare means, and the Kruskal-Wallis test to compare medians. The association between the contraceptive method and the acute painful episodes was evaluated by the Chi-square test. Results The mean age of women with SCD was 28.3 years and 86.6% were mixed or of African-American ethnicity. With respect to the genotypes, 134 women (84.8%) had HbSS genotype, 12 women (7.6%) had hemoglobin SC (HbSC) disease genotype, and 12 (7.6%) were identified with hemoglobinopathy S-beta (S-ß) thalassemia. The mean age of HbSS diagnosis was lower than that of HbSC disease, the less severe form of SCD (p < 0.001). The mean age of menarche was 14.8 ± 1.8 years for HbSS and 12.7 ± 1.5 years for HbSC (p < 0.001). Among women with HbSS who used progestin-only contraception, 16.6% had more than 4 acute painful episodes per year. There was no statistically significant difference when compared with other contraceptive methods. Conclusion With respect to reproduction-associated variables, only the age of the menarche showed delay in HbSS when compared with HbSC. The contraceptive method used was not associated with the frequency of acute painful episodes among the HbSS women.

Objetivo Descrever as variáveis reprodutivas em diferentes genótipos da doença falciforme (DF) e a influência dos métodos contraceptivos na frequência das crises álgicas em mulheres com homozigose da hemoglobina S (HbSS). Métodos Estudo de corte transversal realizado entre setembro de 2015 e abril de 2016 com 158 mulheres com DF atendidas em um centro de hematologia no Nordeste do Brasil. As variáveis reprodutivas dos diferentes genótipos da DF foram avaliadas utilizando-se o teste de análise de variância (ANOVA) para comparação de médias e o teste de Kruskal-Wallis para comparação de medianas. A associação entre o método contraceptivo e a frequência das crises álgicas foi avaliada pelo teste Qui-quadrado. Resultados A idade média das mulheres com DF foi de 28,3 anos e 86,6% eram afrodescentes. Em relação aos genótipos, 134 mulheres (84,8%) tinham genótipo HbSS, 12 mulheres (7,6%) tinham genótipo para doença da hemoglobina SC (HbSC) e 12 (7,6%) foram identificadas com beta talassemia (S-ß). A idade média do diagnóstico de HbSS foi menor do que a da HbSC, sendo esta a forma menos grave da DF (p < 0,001). A idade média da menarca foi de 14,8 ± 1,8 anos para HbSS e de 12,7 ± 1,5 anos para HbSC (p < 0,001). Entre as mulheres com HbSS que fizeram contracepção com progesterona isolada, 16,6% apresentaram mais de 4 episódios de crises álgicas agudas por ano. Não houve diferença estatisticamente significativa quando comparado com outros métodos anticoncepcionais. Conclusão Em relação às variáveis reprodutivas, apenas a idade da menarca apresentou atraso no HbSS em relação ao HbSC. O método anticoncepcional utilizado não foi associado à frequência de crises álgicas entre as mulheres com HbSS.