La Clinica terapeutica · 2025
A Rare Presentation of HLH as acute encephalopathy, fever and Diabetic Ketoacidosis (DKA) followed by massive pulmonary embolism: Diagnostic and Therapeutic Challenges.
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Alharbi Ahmad, El Besh Mohammed, Farrag Naglaa, Kamal Mohamed
Аннотация
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life‑threatening hyperinflammatory syndrome that often presents with nonspecific features, delaying diagnosis and treatment. Although fever, cytopenias, and organomegaly are classic, unusual presentations can obscure recognition. We report an exceptional case of secondary HLH manifesting as severe diabetic ketoacidosis (DKA) with cerebral edema in a young man with type 1 diabetes, later complicated by a massive saddle pulmonary embolism.
CASE PRESENTATION: A 25‑year‑old man with a history of type 1 diabetes mellitus (HbA₁c 10.4%) presented with five days of high‑grade fever (39° C), confusion, agitation, productive cough, and dyspnoea. On admission, he exhibited a high‑anion‑gap metabolic acidosis (pH 7.15, HCO₃⁻ 10 mmol/L), hyperglycaemia (427 mg/dL), leucocytosis (23 × 10³/µL), bilateral lower‑lobe pneumonia on chest CT, and mild cerebral oedema on CT brain. He was managed for DKA and pneumonia with insulin infusion, fluid resuscitation, broad‑spectrum antibiotics, and mechanical ventilation. Despite correction of acidosis, fever persisted, and cultures remained negative. Progressive bicytopenia (Hb 8.5 g/dL; platelets 92 × 10³/µL), hyperferritinemia (2,000 ng/mL), hypertriglyceridemia 337mg/dl-3.805 mmol/l). hepatosplenomegaly, and an HScore of 198 prompted bone marrow biopsy which confirmed hemophagocytosis. High‑dose methylprednisolone and cyclosporine led to dramatic improvement by ICU Day 12. On day 16, he developed hypotension, tachycardia, and hypoxemia; CT pulmonary angiography revealed a saddle pulmonary embolism. Thrombolysis with rTPA and anticoagulation stabilized him, and by discharge his ferritin had declined to 850 ng/mL and cytopenias had normalized.
CONCLUSIONS: This case underscores the diagnostic challenge of HLH presenting as metabolic crisis and highlights the importance of early consideration of HLH in patients with unexplained inflammatory and hematologic derangements. Clinicians should also remain vigilant for thromboembolic complications during HLH treatment.
CASE PRESENTATION: A 25‑year‑old man with a history of type 1 diabetes mellitus (HbA₁c 10.4%) presented with five days of high‑grade fever (39° C), confusion, agitation, productive cough, and dyspnoea. On admission, he exhibited a high‑anion‑gap metabolic acidosis (pH 7.15, HCO₃⁻ 10 mmol/L), hyperglycaemia (427 mg/dL), leucocytosis (23 × 10³/µL), bilateral lower‑lobe pneumonia on chest CT, and mild cerebral oedema on CT brain. He was managed for DKA and pneumonia with insulin infusion, fluid resuscitation, broad‑spectrum antibiotics, and mechanical ventilation. Despite correction of acidosis, fever persisted, and cultures remained negative. Progressive bicytopenia (Hb 8.5 g/dL; platelets 92 × 10³/µL), hyperferritinemia (2,000 ng/mL), hypertriglyceridemia 337mg/dl-3.805 mmol/l). hepatosplenomegaly, and an HScore of 198 prompted bone marrow biopsy which confirmed hemophagocytosis. High‑dose methylprednisolone and cyclosporine led to dramatic improvement by ICU Day 12. On day 16, he developed hypotension, tachycardia, and hypoxemia; CT pulmonary angiography revealed a saddle pulmonary embolism. Thrombolysis with rTPA and anticoagulation stabilized him, and by discharge his ferritin had declined to 850 ng/mL and cytopenias had normalized.
CONCLUSIONS: This case underscores the diagnostic challenge of HLH presenting as metabolic crisis and highlights the importance of early consideration of HLH in patients with unexplained inflammatory and hematologic derangements. Clinicians should also remain vigilant for thromboembolic complications during HLH treatment.