Table 2A.
Calculation of H- Score (6) and Center for Disease Control (CDC) Multisystem Inflammatory Syndrome-Adults (MIS-A) criteria (7). H-Score calculation as per the criteria by Fardet et al. (6) An H-Score of 256 was found in our patient, giving us a 99% probability of hemophagocytic lymphohistiocytosis (HLH).
| Serial no | Parameters | Scoring (CUT OFF) used in H-Score calculation (6) | Our patient profile (score) | MIS-A Criterion (as per CDC) (7) |
|---|---|---|---|---|
| 1 | Known underlying immunosuppression | 0 (no) or 18 (yes) | Yes (18) | • 1. Clinical Criteria: Documented fever (≥38.0 C) for ≥24 h prior to or within the first THREE days of hospitalization along with atleast THREE of the following clinical criteria including one primary clinical criterion. |
| 2 | Temperature (°C) | 0 (<38.4), 33 (38.4–39.4), or 49 (>39.4) | 39.3°C (33) | • 1A: Primary clinical criteria • 1.Severe cardiac illness Includes myocarditis, pericarditis, coronary artery dilatation/aneurysm, or new-onset right or left ventricular dysfunction (LVEF <50%), 2nd/3rd degree A-V block, or ventricular tachycardia. • 2.Rash AND non-purulent conjunctivitis. |
| 3 | Organomegaly | 0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly) | Hepatomegaly (23) | • 1B: Secondary clinical criteria
• 1.New-onset neurologic signs and symptoms include encephalopathy in a patient without prior cognitive impairment, seizures, meningeal signs, or peripheral neuropathy. |
| 4. | Number of cytopenias | 0 (1 lineage), 24 (2 lineages), or 34 (3 lineages) | 3 lineages (34) | • 2.Shock or hypotension not attributable to medical therapy. • 3.Abdominal pain, vomiting, or diarrhea • 4. Thrombocytopenia (platelet count < 150,000/ microliter)* |
| 5. | Ferritin (ng/mL) | 0 (<2,000), 35 (2,000–6,000), or 50 (>6,000) | 4360 ng/ml (35) | • 2. Laboratory evidence • 2A. Elevated levels of at least TWO of the following: C-reactive protein, ferritin, IL-6, erythrocyte sedimentation rate, procalcitonin* |
| 6. | Triglyceride (mmol/L) | 0 (<1.5), 44 (1.5–4), or 64 (>4) | 950 mg/dL = 10.7 mmol/L (64) | • 2B. A positive SARS-CoV-2 test for current or recent infection by RT-PCR, serology, or antigen detection* |
| 7. | Fibrinogen (gm/L) | 0 (>2.5) or 30 (≤ 2.5) | ≤ 250 mg/dL = ≤ 2.5 g/L (30) | |
| 8. | Serum Aspartate transaminase (IU/L) | 0 (<30) or 19 (≥30) | 11062 U/L (19) | |
| 9. | Hemophagocytosis features on bone marrow aspirate | 0 (no) or 35 (yes) | No (0) | |
| 10. | Total | 256 points, giving us a 99% probability of HLH. (Cut off is 169 points) |
*Our patient fulfilled (bold and italicized) 1. One secondary clinical criterion– thrombocytopenia. 2. Two laboratory criteria. But sepsis could not be excluded as defined by CDC for MIS-A in our patient since he developed urinary tract infection. |