Linfohistiocitose hemofagocítica pós-transplante de células-tronco hematopoiéticas ­ relato de caso / Post-hematopoietic stem cell transplant hemophagocytic lymphohistiocytosis ­ case report

A linfohistiocitose hemofagocítica (LHH) é uma condição de inflamação excessiva causada por uma desregulação do sistema imune. As manifestações clínicas são inespecíficas e incluem febre persistente, organomegalia, disfunção hepática, coagulopatia, citopenias, hiperferritinemia e hipertrigliceridemia. Pode ser classificada em primária/familiar ou secundária/adquirida. A LHH pós-transplante de células-tronco hematopoiéticas, um subtipo raro de LHH secundária, apresenta alta mortalidade e é de difícil diagnóstico e manejo. O relato de caso de uma criança de quatro anos que desenvolveu LHH pós-transplante de células-tronco hematopoiéticas alogênico ilustra os desafios desta condição, desde a apresentação clínica, que pode mimetizar outras condições pós-transplante, até a escolha do melhor tratamento

Hemophagocytic lymphohistiocytosis (HLH) is a condition of excessive inflammation caused by dysregulation of the immune system. Clinical manifestations are inespecific and include unremitting fever, organomegaly, liver dysfunction, coagulopathy, cytopenias, hyperferritinemia and hypertriglyceridemia. LHH can be classified as primary/familial or secondary/acquired. Post-hematopoietic stem cell transplantation HLH, a rare subtype of secondary HLH, presents high mortality and is especially difficult to diagnose and manage. A case report of a four-year-old child who developed HLH after allogeneic hematopoietic stem cell transplantation illustrates the challenges of this condition, from clinical presentation, which can mimic other post-transplant conditions, to choosing the best treatment

Does low serum TSH within the normal range have negative impact on physical exercise capacity and quality of life of healthy elderly people?

Objective Investigate the differences in cardiopulmonary (CP) capacity and Quality of Life (QOL) between healthy elderly (≥ 65 years) with different TSH levels (< 1.0 and ≥ 1.0 μIU/mL) both within the normal range. Also, evaluate the effects of TSH elevation on CP test and QOL, by administering methimazole to subjects with initial lower-normal TSH, in order to elevate it to superior-normal limit. Materials and methods Initially, a cross-sectional study was performed to compare CP capacity at peak exercise and QOL (using WHOQOL-OLD questionnaire) between healthy seniors (age ≥ 65 years) with TSH < 1.0 μIU/mL vs. TSH ≥1.0 μIU/mL. In the second phase, participants with TSH < 1.0 μIU/mL were included in a non-controlled-prospective-interventional study to investigate the effect of TSH elevation, using methimazole, on QOL and CP capacity at peak exercise. Results From 89 elderly evaluated, 75 had TSH ≥ 1 μIU/mL and 14 TSH < 1 μIU/mL. The two groups had similar basal clinical characteristics. No difference in WHOQOL-OLD scores was observed between groups and they did not differ in terms of CP function at peak exercise. QOL and CP variables were not correlated with TSH levels. Twelve of 14 participants with TSH < 1.0 μIU/mL entered in the prospective study. After one year, no significant differences in clinical caracteristics, QOL, and CP variables were detected in paired analysis before and after methimazole intervention. Conclusions We found no differences in CP capacity and QOL between health elderly with different TSH levels within normal range and no impact after one year of methimazole treatment. More prospective-controlled-randomized studies are necessary to confirm or not the possible harm effect in normal low TSH.

Does low serum TSH within the normal range have negative impact on physical exercise capacity and quality of life of healthy elderly people?

OBJECTIVE: Investigate the differences in cardiopulmonary (CP) capacity and Quality of Life (QOL) between healthy elderly (≥ 65 years) with different TSH levels (< 1.0 and ≥ 1.0 µIU/mL) both within the normal range. Also, evaluate the effects of TSH elevation on CP test and QOL, by administering methimazole to subjects with initial lower-normal TSH, in order to elevate it to superior-normal limit. MATERIALS AND METHODS: Initially, a cross-sectional study was performed to compare CP capacity at peak exercise and QOL (using WHOQOL-OLD questionnaire) between healthy seniors (age ≥ 65 years) with TSH < 1.0 µIU/mL vs. TSH ≥1.0 µIU/mL. In the second phase, participants with TSH < 1.0 µIU/mL were included in a non-controlled-prospective-interventional study to investigate the effect of TSH elevation, using methimazole, on QOL and CP capacity at peak exercise. RESULTS: From 89 elderly evaluated, 75 had TSH ≥ 1 µIU/mL and 14 TSH < 1 µIU/mL. The two groups had similar basal clinical characteristics. No difference in WHOQOL-OLD scores was observed between groups and they did not differ in terms of CP function at peak exercise. QOL and CP variables were not correlated with TSH levels. Twelve of 14 participants with TSH < 1.0 µIU/mL entered in the prospective study. After one year, no significant differences in clinical caracteristics, QOL, and CP variables were detected in paired analysis before and after methimazole intervention. CONCLUSIONS: We found no differences in CP capacity and QOL between health elderly with different TSH levels within normal range and no impact after one year of methimazole treatment. More prospective-controlled-randomized studies are necessary to confirm or not the possible harm effect in normal low TSH.