Figure 1.
The distribution of HLH-defining features reveals significant overlap between patients with uncomplicated HMs and patients with malignancies complicated by HLH, regardless of clinical suspicion for HLH. Normal range and the diagnostic threshold level from the HLH-2004 study (used for FHL) are shown in patients examined as routine at HM diagnosis (left bars) and patients tested due to HLH suspicion (right bars). These values are the preliminary measurements at the initial presentation of HLH/malignancy. The distribution of maximally pathologic values during the index encounter is shown in supplemental Figure 2. (A) Distribution of the inflammatory markers. The values of sCD25 between HM-HLH with suspected HLH and those evaluated as routine were not significantly different (P = .09), whereas the ferritin values were (P = .016). (B) Distribution of the blood lineages. (C) Distribution of other markers. (D) Categorical parameters. The statistics of the quantitative parameters were analyzed by using the Mann-Whitney U test. The statistics of the categorical parameters were analyzed with Fisher’s exact test. Values shown were available for >90% of the patients. NK activity was available for only 4% of the patients and therefore is not shown. HM = patients with uncomplicated HMs; HM-HLH = patients fulfilling 5 of 8 HLH-2004 diagnostic criteria.*P < .05, **P < .01, ***P < .001 ****P < .0001. ANC, absolute neutrophil count; Hb, hemoglobin; N/A, not available; ns, not significant; PLT, platelets; Tg, triglycerides.

The distribution of HLH-defining features reveals significant overlap between patients with uncomplicated HMs and patients with malignancies complicated by HLH, regardless of clinical suspicion for HLH. Normal range and the diagnostic threshold level from the HLH-2004 study (used for FHL) are shown in patients examined as routine at HM diagnosis (left bars) and patients tested due to HLH suspicion (right bars). These values are the preliminary measurements at the initial presentation of HLH/malignancy. The distribution of maximally pathologic values during the index encounter is shown in supplemental Figure 2. (A) Distribution of the inflammatory markers. The values of sCD25 between HM-HLH with suspected HLH and those evaluated as routine were not significantly different (P = .09), whereas the ferritin values were (P = .016). (B) Distribution of the blood lineages. (C) Distribution of other markers. (D) Categorical parameters. The statistics of the quantitative parameters were analyzed by using the Mann-Whitney U test. The statistics of the categorical parameters were analyzed with Fisher’s exact test. Values shown were available for >90% of the patients. NK activity was available for only 4% of the patients and therefore is not shown. HM = patients with uncomplicated HMs; HM-HLH = patients fulfilling 5 of 8 HLH-2004 diagnostic criteria.*P < .05, **P < .01, ***P < .001 ****P < .0001. ANC, absolute neutrophil count; Hb, hemoglobin; N/A, not available; ns, not significant; PLT, platelets; Tg, triglycerides.

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