A 57-year-old previously healthy fisherman was admitted in fulminant pneumococcal septic shock, with disseminated intravascular coagulation, requiring aggressive management including bilateral below-knee amputations for ischaemic necrosis. He began to recover and was discharged for rehabilitation, however during his convalescence was found to be hypercalcaemic. No malignancy was found on CT scan, but it was noted that his spleen was absent, replaced by a 4 cm smooth-walled, fluid-filled lesion. This was unexpected as an ultrasound in intensive care 10 weeks previously had demonstrated a normal spleen. Functional hyposplenism was confirmed on a peripheral blood film with evidence of target cells, spherocytes and Howell-Jolly bodies. A diagnosis of autosplenectomy complicating pneumococcal sepsis was therefore made, of which there is just one case previously reported. The patient continues to recover well and was discharged on penicillin prophylaxis after receiving vaccinations for hyposplenism.
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http://dx.doi.org/10.1136/bcr-2017-219561 | DOI Listing |
Cureus
October 2024
Basra Hematology Center, Basra Health Directorate, Basra, IRQ.
Background Hemoglobinopathies, such as sickle cell disease (SCD), are inherited disorders of hemoglobin (Hb) synthesis. SCD presents with complex clinical manifestations, including anemia, painful episodes, and organ damage due to recurrent vaso-occlusion. The spleen is one of the first organs affected in SCD patients, with hyposplenism typically occurring by age five.
View Article and Find Full Text PDFAm J Case Rep
September 2024
Department of Surgery, The Brooklyn Hospital Center, Icahn School of Medicine at Mount Sinai, New York City, NY, USA.
Clin Rheumatol
October 2024
Department of Medicine, Division of Rheumatology, Queen's University, Kingston, ON, Canada.
Mediterr J Hematol Infect Dis
May 2024
Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.
Background: Sickle cell disease (SCD) is a major public health issue worldwide with high morbidity and mortality. SCD SD Punjab is the third most common genotype of SCD in Oman and is associated with several serious complications. The aim of the study is to establish the clinical and laboratory features of SCD patients with SD double heterozygotes and study the impact of haemoglobin F, hydroxyurea, and other modulators on the disease severity.
View Article and Find Full Text PDFCureus
March 2024
Hematology and Medical Oncology, Interfaith Medical Center, Brooklyn, USA.
Sickle cell disease is a hereditary red blood cell disorder characterized by hemolytic anemia, particularly in association with stress. As they grow, most children with sickle cell anemia undergo auto-splenectomy, making them vulnerable to serious infections. Patients with sickle cell disease infected with the SARS-CoV-2 virus are reported to have an increased risk for hospitalization, thrombosis, and other complications compared to non-sickle cell patients.
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