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(a) CT abdomen on admission reveals edematous gallbladder which was later found to have evidence of Histoplasma capsulatum. (b, c) Bone marrow aspirate reveals hemophagocytosis. (d) GMS stain of bone marrow core biopsy positive for histoplasmosis.
Source publication
Background:
Hemophagocytic lymphohistiocytosis (HLH) was originally described in pediatric patients presenting with fever, hepatosplenomegaly, and blood cell abnormalities. Later, HLH was recognized to occur in adults, often associated with hematologic malignancies or serious infections.
Conclusion:
Patients presenting with HLH are critically il...
Citations
... Acquired HLH has recently been increasingly recognized in patients with hematologic diseases and rheumatic diseases, but it is not easy to distinguish the two causes. Secondary HLH is a wellrecognized complication of rheumatic diseases, such as systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD), systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), and Sjögren syndrome (7)(8)(9)(10)(11)(12). In addition, secondary HLH in the setting of malignancy is commonly reported in adults and occurs in an estimated 1% of adult hematologic malignancies (13). ...
Background
Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease of immune hyperactivation that arises in the context of infectious, inflammatory, or neoplastic triggers. The aim of this study was to establish a predictive model for the timely differential diagnosis of the original disease resulting in HLH by validating clinical and laboratory findings to further improve the efficacy of therapeutics for HLH.
Methods
We retrospectively enrolled 175 secondary HLH patients in this study, including 92 patients with hematologic disease and 83 patients with rheumatic disease. The medical records of all identified patients were retrospectively reviewed and used to generate the predictive model. We also developed an early risk score using multivariate analysis weighted points proportional to the β regression coefficient values and calculated its sensitivity and specificity for the diagnosis of the original disease resulting in HLH.
Results
The multivariate logistic analysis revealed that lower levels of hemoglobin and platelets (PLT), lower levels of ferritin, splenomegaly and Epstein−Barr virus (EBV) positivity were associated with hematologic disease, but young age and female sex were associated with rheumatic disease. The risk factors for HLH secondary to rheumatic diseases were female sex [OR 4.434 (95% CI, 1.889-10.407), P =0.001], younger age [OR 6.773 (95% CI, 2.706-16.952), P<0.001], higher PLT level [OR 6.674 (95% CI, 2.838-15.694), P<0.001], higher ferritin level [OR 5.269 (95% CI, 1.995-13.920), P =0.001], and EBV negativity [OR 27.656 (95% CI, 4.499-169.996), P<0.001]. The risk score included assessments of female sex, age, PLT count, ferritin level and EBV negativity, which can be used to predict HLH secondary to rheumatic diseases with an AUC of 0.844 (95% CI, 0.836~0.932).
Conclusion
The established predictive model was designed to help clinicians diagnose the original disease resulting in secondary HLH during routine practice, which might be improve prognosis by enabling the timely treatment of the underlying disease.
Hemophagocytic lymphohistiocytosis (HLH) secondary to Histoplasma capsulatum is rare, impacting <1% globally, with a mortality rate of up to 31%. Herein, we present a rare case of HLH secondary to H capsulatum, affecting a 57-year-old female with rheumatoid arthritis. Extensive investigations were unrevealing and despite broad-spectrum antibiotics, her condition worsened, leading to respiratory failure requiring extracorporeal membrane oxygenation (ECMO) support, shock requiring multiple vasopressors, and acute kidney injury (AKI) requiring hemodialysis. Diagnosis confirmed disseminated histoplasmosis (DHP), prompting Amphotericin B and methylprednisolone treatment, resulting in significant improvement and discharge with posaconazole therapy. Secondary HLH, primarily arising from severe infections like DHP, is discussed. Limited research exists on this condition in human immunodeficiency virus (HIV)-seronegative individuals. Diagnosis involves HLH-2004 and HScore criteria. Managing histoplasmosis-associated HLH remains challenging due to multiorgan failure risks and treatment complexities and needs further research.
An autoimmune disease is described as a disease caused by an abnormal immune response. Autoimmune diseases (such as type 1 diabetes (T1D), multiple sclerosis (MS), and systemic lupus erythematosus (SLE)) are among the most common chronic illnesses caused by a dysregulated inflammatory response against self-antigens. Autoimmunity affects about 5% of the general population, and the geoepidemiology of these diseases becomes more complicated when certain factors like age, gender, ethnicity, and other demographic characteristics are considered. Autoimmunity in detail was discussed in this chapter.KeywordsAutoimmunityAbnormal immune responseType 1 diabetesMultiple sclerosisSystemic lupus erythematosus
