Author(s)

Lydie Ocini Ngolet^1*, Vulliez Renucci Nkounkou¹, Blaise Atipo Ibara², Alexis Elira Dokekias¹

¹Department of Medical Hematology, University Hospital of Brazzaville, Brazzaville, The Republic of the Congo.
²Department of Internal Medicine and Gastroenterology Unit, University Hospital of Brazzaville, Brazzaville, The Republic of the Congo.

Background: There are no consensual diagnosis criteria to make diagnosis of sickle cell intrahepatic cholestasis and identify its 2 variants. Here we delineate the frequency of the complication in its severe form and describe its clinical features. Methods: We included in the study all the patients with sickle cell disease who were presenting fever, jaundice, pain in the upper right quadrant and filling up the biological criteria of the severe sickle cell intrahepatic cholestasis (serum direct bilirubin ≥ 78.5 mg/dL). Patients with evidence of viral hepatitis or gallstones were not included in the study. Results: Sixty-two patients with an average age of 21.5 years (range 18 and 25 years) meet the inclusion criteria, minimum serum direct bilirubin: 82 versus 528 for the maximum. The liver cytolysis was major and ranged from 310 to 1550 UI/L. Forty-nine patients (83.9%) did not develop any organ failure. The symptoms and biological abnormalities resolved with supportive treatment within 7 days. The global lethality was 16.1% associated with liver failure. Conclusion: Severe sickle cell intrahepatic cholestasis is more frequent than we thought and was mainly represented by borderline clinical feature. Early intervention may reduce the progression to organ failure stage and ultimate death.

Sickle Cell Disease, Serum Direct Bilirubin, Borderline Clinical Feature

Ngolet, L. , Nkounkou, V. , Ibara, B. and Dokekias, A. (2020) Severe Variant of Sickle Cell Intrahepatic Cholestasis a Difficult Approach Diagnosis Clinical Presentation and Outcome in Adult Population in the Congo. Open Journal of Blood Diseases, 10, 41-47. doi: 10.4236/ojbd.2020.102005.