Konstantinos Kalliamvakos

konstantinos.kalliamvakos@cambio.se

Cambio CDS

To assist clinicians in the diagnosis of reactive hemophagocytic syndrome.

<div><p>The HScore can be used to estimate an individual&#39;s risk of having reactive hemophagocytic syndrome.</p><p><strong>Formula</strong></p><p>Addition of the selected points:</p><table><thead><tr style="text-align:left;"><th>Variable</th><th></th><th>Points</th></tr></thead><tbody><tr><td rowspan=2>Known underlying immunosuppression*</td><td>No</td><td>0</td></tr><tr><td>Yes</td><td>18</td></tr><tr><td rowspan=3>Temperature, °F (°C)</td><td>&lt;101.1 (&lt;38.4)</td><td>0</td></tr><tr><td>101.1–102.9 (38.4-39.4)</td><td>33</td></tr><tr><td>&gt;102.9 (&gt;39.4)</td><td>49</td></tr><tr><td rowspan=3>Organomegaly</td><td>No</td><td>0</td></tr><tr><td>Hepatomegaly or splenomegaly</td><td>23</td></tr><tr><td>Hepatomegaly and splenomegaly</td><td>38</td></tr><tr><td rowspan=3>Number of cytopenias**</td><td>1 lineage</td><td>0</td></tr><tr><td>2 lineages</td><td>24</td></tr><tr><td>3 lineages</td><td>34</td></tr><tr><td rowspan=3>Ferritin, ng/mL (μg/L)</td><td>&lt;2,000</td><td>0</td></tr><tr><td>2,000–6,000</td><td>35</td></tr><tr><td>&gt;6,000</td><td>50</td></tr><tr><td rowspan=3>Triglyceride, mg/dl (mmol/l)</td><td>&lt;132.7 (&lt;1.5)</td><td>0</td></tr><tr><td>132.7-354 (1.5–4)</td><td>44</td></tr><tr><td>&gt;354 (&gt;4)</td><td>64</td></tr><tr><td rowspan=2>Fibrinogen, mg/dl (g/L)</td><td>&gt;250 (&gt;2.5)</td><td>0</td></tr><tr><td>≤250 (≤2.5)</td><td>30</td></tr><tr><td rowspan=2>AST, U/l</td><td>&lt;30</td><td>0</td></tr><tr><td>≥30</td><td>19</td></tr><tr><td rowspan=2>Hemophagocytosis features on bone marrow aspirate</td><td>No</td><td>0</td></tr><tr><td>Yes</td><td>35</td></tr></tbody></table><p>*HIV positive or receiving long‐term immunosuppressive therapy (i.e., glucocorticoids, cycloSPORINE, azaTHIOprine).</p><p>**Defined as hemoglobin ≤9.2 g/dL (≤5.71 mmol/l) and/or WBC ≤5,000/mm³ and/or platelets ≤110,000/mm³.</p><p><strong>Interpretation</strong></p><table style=><thead><tr style="text-align:left;"><th>HScore</th><th></th><th></th><th>Probability of hemophagocytic syndrome</th></tr></thead><tbody><tr><td>≤90</td><td></td><td></td><td>&lt;1%</td></tr><tr><td>91-100</td><td></td><td></td><td>~1%</td></tr><tr><td>101-110</td><td></td><td></td><td>1-3%</td></tr><tr><td>111-120</td><td></td><td></td><td>3-5%</td></tr><tr><td>121-130</td><td></td><td></td><td>5-9%</td></tr><tr><td>131-140</td><td></td><td></td><td>9-16%</td></tr><tr><td>141-150</td><td></td><td></td><td>16-25%</td></tr><tr><td>151-160</td><td></td><td></td><td>25-40%</td></tr><tr><td>161-170</td><td></td><td></td><td>40-54%</td></tr><tr><td>171-180</td><td></td><td></td><td>54-70%</td></tr><tr><td>181-190</td><td></td><td></td><td>70-80%</td></tr><tr><td>191-200</td><td></td><td></td><td>80-88%</td></tr><tr><td>201-210</td><td></td><td></td><td>88-93%</td></tr><tr><td>211-220</td><td></td><td></td><td>93-96%</td></tr><tr><td>221-230</td><td></td><td></td><td>96-98%</td></tr><tr><td>231-240</td><td></td><td></td><td>98-99%</td></tr><tr><td>≥241</td><td></td><td></td><td>&gt;99%</td></tr></tbody></table><p>Note: the best cutoff value for HScore was 169, corresponding to a sensitivity of 93%, specificity of 86%, and accurate classification of 90% of the patients.</p></div>

1. Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol. 2014;66(9):2613-20.

EVALUATION.hscore.v0, OBSERVATION.body_temperature.v2, OBSERVATION.lab_test-ferritin.v0, OBSERVATION.lab_test-lipids.v1, OBSERVATION.lab_test_fibrinogen.v0, OBSERVATION.lab_test-liver_function.v1