Spontaneous Subgaleal Hematoma in a Sickle Cell Disease Patient: A Case Report

Abstract

Sickle cell disease (SCD) is one of the most commonly inherited conditions on the Arabian Peninsula. We report a case of a 17-yearold boy, who had previously been diagnosed with SCD and glucose-6-phosphate dehydrogenase (G6PD) deficiency and recently presented to our hospital with spontaneous subgaleal hematoma (SGH), which was managed conservatively. We also present a literature review on the topic of spontaneous intra-and/or extra-cranial bleeds.

Recurrent Nontraumatic Subgaleal Hematomas in a Pediatric Patient With Sickle Cell Disease

“…Conservative management with adequate hydration, pain management, and imaging to rule out ischemic stroke facilitates timely discharge with close outpatient follow-up. Our management was in line with other published case reports of subgaleal hematoma 2,10,11 . Surgical evacuation and embolization is rarely required, although it has been reported in a case due to recurrence (>2 episodes) and expansion of hematoma 12 .…”

“…Our management was in line with other published case reports of subgaleal hematoma. 2,10,11 Surgical evacuation and embolization is rarely required, although it has been reported in a case due to recurrence ( > 2 episodes) and expansion of hematoma. 12 Patients with SCD may have a propensity for bleeding in the presence of bone infarction, as evidenced by the development of recurrent subgaleal hematomas in our patient with skull infarction.…”

Acute soft head syndrome in a teenager with sickle cell anemia: A case report

“…The index patient presented with typical manifestation of ASHS in agreement to other reported cases across countries and ethnicities 12,30 . There is no any confirmed predilection of specific skull bone, however, of the majority of reported cases, frontal bone appears to be the most commonly affected for reasons which remains largely unknown 25 . Noteworthy, the available case reports indicates that patients with ASHS are often boys, if not all, with debut typically at puberty, the age range of 11–20 years, the majority being 16 years as the index case 25,31–33 .…”

“…The expression of 15%–40% of HbF typically sets the physiological and biochemical threshold for preventing sickling in SCD 22 . Thus, the index patients had Hb of 9.8 g/dL and HbF 19.50%, which is comparable to several reported cases, which had Hb levels ranging from 6.0–11 g/gL, and HbS ranging from 65.0%–90.0% respectively and comparable HbF levels 11,25–27 . The expression of HbF and its distribution in RBCs is regulated by elements linked to β‐globulin complex and are associated with specific β‐globin haplotype and trans ‐acting elements associated with BCL11A and HBS1L‐MYB intergenic region on chromosome 2p16 and 6q23, respectively 22 .…”

“… 22 Thus, the index patients had Hb of 9.8 g/dL and HbF 19.50%, which is comparable to several reported cases, which had Hb levels ranging from 6.0–11 g/gL, and HbS ranging from 65.0%–90.0% respectively and comparable HbF levels. 11 , 25 , 26 , 27 The expression of HbF and its distribution in RBCs is regulated by elements linked to β‐globulin complex and are associated with specific β‐globin haplotype and trans ‐acting elements associated with BCL11A and HBS1L‐MYB intergenic region on chromosome 2p16 and 6q23, respectively. 22 BCL11A is a critical transcriptional factor expressed in hematopoietic tissues which control HbF switching through interaction with GATA1; a DNA‐binding zinc finger motif.…”