PurposeHereditary angioedema (HAE) is a rare genetic disease with hyperactivated contact and kallikrein-kinin systems leading to bradykinin (BK) release and edema. SARS-CoV-2 infection results in inflammatory exacerbation. C1 inhibitor (C1-INH) deficiency could aggravate clinical outcomes, with HAE patients at a greater risk of adverse outcomes of COVID-19, however, data are still limited. Our aim was to characterize the course and severity of COVID-19 in patients with HAE.MethodsLatin American HAE reference centers evaluated SARS-CoV-2 infection in this population. Patients with confirmed diagnosis of HAE with (HAE-C1-INH) or without C1-INH deficiency (HAE-nC1-INH) were included. HAE symptomatology and the course of COVID-19 were characterized with the application of a questionnaire. Results66 patients from 10 countries (HAE-C1-INH 80,3%; HAE-nC1-INH 19.6%) were reported with SARS-CoV-2 infection. Comorbidities were absent in 69.7% of the patients and obesity present in 12.1%. Attacks occurred in 45.5% of patients with HAE during SARS-CoV-2 infection. Long term prophylaxis was reported in 52% (34/66) of HAE patients. Complete cure was observed in 61 patients (92.4%), pulmonary sequelae in 4 and death in one HAE-C1-INH patient. The cause of death was septic shock secondary to bacterial pulmonary coinfection. Disease progression was not impacted by sex, therapy or type of HAE (p = 0.803). ConclusionAttacks occurred in almost half of HAE patients suggesting that SARS-CoV-2 infection is a trigger. HAE did not represent a risk factor for a worse outcome of COVID-19, even in use of androgens.
Objectives: Plasma cell dyscrasias are diseases characterized by clonal proliferation and accumulation of cells producing monoclonal immunoglobulins. These diseases have not been studied in our region and we don't know if their behavior is similar to that reported in the literature. That's why we evaluated multiple characteristics in southern Colombia. Methods: analytical cross-sectional study of patients with confirmed diagnosis of a plasma cell dyscrasias were included. Results: 60 patients included in our study, 65% were men, with an average age of 58.8 years (CI 96% 55.8 -61.93). Bone pain was the most frequent symptom (88%). The most frequent dyscrasia was multiple myeloma and in these patients we found a high percentage of hemoglobin less than 10 mg/dl, creatinine greater than 2 mg/dl and serum calcium higher than 11 mg/dl (77%, 38% and 37 %, respectively). Half of the patients had a time course of symptoms greater than 4 months and 43% had plasma cells in bone marrow greater than 60%. 65% of patients had elevated levels of serum B2-microglobulin (> 5.5 mg/L) and in-hospital mortality was 15%. We found a statistically significant association between mortality and gender (PR 6.5) and between mortality and hemoglobin (p = 0.039).
Conclusion:Patients with plasma cell dyscrasia in southern Colombia are younger, consult late, in an advanced stage of their disease, with greater renal damage, hypercalcemia and anemia than reported in the literature, also a high tumor burden due to high plasma cell infiltration into bone marrow and high values of serum B2-microglobulin.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.