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Hemophagocytic lymphohistiocytosis (HLH) is a progressive and potentially life-threatening disorder. It is classified into primary and secondary HLH. The objective of our study was to determine the outcome of primary and secondary HLH in pediatric and adult patients based on HScore and treatment modality. We conducted a retrospective analysis done from July 2010 to June 2020. Variables analyzed included age, gender and history of death in siblings. HScore was used for disease classification while clinical and laboratory findings which were required to fulfill the HScore diagnostic criteria were also recorded. Continuous variables were summarized as median and categorical variables as frequencies and percentages. Categorical variables were compared using chi-square test and Fisher Exact test. Significance of different variables between primary and secondary HLH was calculated using independent-samples t test. A P value of < .05 was taken as significant. A total of 51 patients were included in the analysis (41 in primary and 10 in secondary HLH group). In primary HLH, 36 patients were in the pediatric age group and 12.2% had a history of death in sibling. All 41 patients had increased ferritin and decreased fibrinogen levels. The overall survival in primary HLH was 44%. In the secondary HLH group, viral infections were the most common etiology and ferritin was increased as well. The overall survival in secondary HLH was 60%. The median survival was 15 ± 4.8 months. The overall survival of both groups combined was 53%. Primary HLH should be considered in pediatric patients who present with pancytopenia and hepatosplenomegaly. In centers where genetic testing is not available, HScore along with serum ferritin and fibrinogen is a good substitute for disease classification.

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BACKGROUND: The mini-Clinical Evaluation Exercise (mini-CEX) and direct observation of procedural skills (DOPS) are reliable tools for work-based assessment of medical trainees. Tools of this type do not yet exist for evaluation of practical laboratory skills of pathology residents. OBJECTIVE: We developed and piloted a 9-item instrument for direct observation of laboratory skills (DOLS). METHODS: We used the DOLS tool with 10 hematopathology residents (PGY-1 to PGY-5) from Aga Khan University. Each resident was evaluated by 3 faculty members in the laboratory during 4 separate encounters using the DOLS instrument. We assessed construct validity, interrater reliability and G coefficient, feasibility of using DOLS, and learner satisfaction. RESULTS: A total of 120 encounters were observed with a mean score (±1 SD) of 56.7% (±12.44). Assessment scores moderately correlated with the number of laboratory procedures previously performed by participants (r = 0.658 and 0.641; P = .0001) and with PGY level. Interrater reliability ranged between 0.47 and 0.96. Cohen's d was 1.64. Residents accounted for a large component of estimated variance (73%), suggesting DOLS can differentiate residents' laboratory skills; variance associated with assessors was small (0.01%). Residents reported being satisfied with the tool. Mean time (±1 SD) taken for observing and feedback was 17.89 ± 5.89 minutes. CONCLUSIONS: The new DOLS instrument could provide reliable scores for observing laboratory skills. Residents were satisfied with the tool, and rating times make the tool feasible for formative assessments.

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