Hair-on-end appearance in a case of thalassemia intermedia

Ballikar, Riya; Redkar, Neelam; Patil, Meenakshi Amit; Pillai, Rajit

doi:10.1136/bcr-2012-008095

Cited by 8 publications

(2 citation statements)

References 3 publications

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“…HOE sign is majorly seen in children over the age of 5 years of age with hemolytic anemias, that is; thalassemia and sickle cell disease. 8 , 9 Our case was of a 9 year old girl in whom the diagnosis of SCD had been missed several times, giving room for this musculoskeletal complication to arise. HOE sign has been reported to have a prevalence of 5% in skull radiographs of 194 patients with sickle cell anemia.…”

Section: Discussionmentioning

confidence: 99%

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease

Ogwang¹,

Odongo²,

Namusisi³

et al. 2022

IMCRJ

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Background The hair-on-end (HOE) sign is a rare finding seen in the diploic space on skull radiographs, computed tomography (CT) and magnetic resonance imaging (MRI) with the appearance of long, thin vertical striations of calcified spicules perpendicular to the bone surface that looks like hair standing on end. It is classically seen in children/adolescents with hemolytic anemias, in particular, thalassemia major and sickle cell disease. Here, we present a 9-year-old Ugandan girl who presented with stroke in whom head CT demonstrated cerebral intraparenchymal hemorrhage and multiple infarcts on the left with HOE sign. Hb electrophoresis confirmed the diagnosis of sickle cell anemia. Case Summary We present a 9-year-old Ugandan girl who presented with an unexplained stroke that preceded an episode of acute headache, vomiting, followed by focal convulsions and altered consciousness. Clinical findings revealed severe pallor of the conjunctivae and mild scleral icterus. CT demonstrated right cerebral intraparenchymal hemorrhage, multiple high cerebral infarcts on the right and evidence of extra medullary hematopoiesis with a classical HOE sign. Hemoglobin (Hb) electrophoresis confirmed sickle cell disease (SCD). The child was then initiated on hydroxyurea, antibiotics, analgesics and intravenous fluids. She improved and was discharged 16 days later. Follow-up of the child was uneventful. Conclusion HOE sign is a complication of chronic hemolysis usually seen in patients with thalassemia and sickle cell anemia. It is a rare finding that clinicians should be well conversant with, especially in Africa where sickle cell disease is common.

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Section: Discussionmentioning

confidence: 99%

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease

Ogwang¹,

Odongo²,

Namusisi³

et al. 2022

IMCRJ

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“…Although di cult, it is nevertheless possible to distinguish, in skeletal samples from archaeological contexts, individuals with thalassemia intermedia or minor who had time to develop pathognomonic features of the disease on their bones (Lagia et al 2007;Balikar et al 2013). On the contrary, the detection of b-thalassemia in the skeletal remains of children is far more complex, as individuals affected by the homozygous form of thalassemia would not have survived long enough to develop typical features such as «hair-on-end» or marrow hyperplasia of the facial bones, except in rare cases (Lewis 2012).…”

Section: Introductionmentioning

confidence: 99%

A new approach for the diagnosis of b-thalassemia in archaeological contexts: the relationship between congenital anaemia and dentinogenesis defects through micro-CT

Viva

Vincenti

Pepe

et al. 2023

Preprint

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The application of radiology as an aid for paleoanthropological analyses is seeing a widespread increase. The aim of this study is to examine a number of radiological dental aspects related to hereditary haemoglobinopathies, such as b-thalassemia, on the human remains of a past community by way of micro-CT. A sample from the early medieval (10th-11th century) archaeological site of Vetricella (Scarlino, Grosseto), located in an area historically affected by malaria and part of the nEU-Med Advanced ERC project, was selected. Macroscopic paleopathological analysis revealed features typical of b-thalassemia. As a result, the skeletal remains underwent CT and micro-CT examination. CT analysis of the ribs, as demonstrated in previous studies, confirmed the initial hypothesis of the presence of b-thalassemia in the group. The dental micro-CT carried out on a sample of 7 deciduous incisors belonging to 7 sub-adult individuals allowed observing for the first time a direct relationship between the degrees of b-thalassemia recorded on the skeletons and dentinogenesis defects, identifying also a new radiological evidence which will be termed as «iris-like» appearance. A sample of three individuals from a coeval, non-thalassemic community was used for comparative purposes. These observations constitute a new approach to the diagnosis of b-thalassemia in archaeological contexts, providing an additional tool for differential diagnoses while also furthering our knowledge of the natural history of this disease.

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Periosteum: Characteristic imaging findings with emphasis on radiologic-pathologic comparisons

et al. 2014

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The periosteum covers most bone structures. It has an outer fibrous layer and an inner cambial layer that exhibits osteogenic activity. The periosteum is a dynamic structure that plays a major role in bone modeling and remodeling under normal conditions. In several disorders such as infections, benign and malignant tumors, and systemic diseases, the osteogenic potential of the periosteum is stimulated and new bone is produced. The newly formed bone added onto the surface of the cortex adopts various configurations depending on the modalities and pace of bone production. Our aim here is to describe the anatomy, histology, and physiology of the periosteum and to review the various patterns of periosteal reaction with emphasis on relations between radiological and histopathological findings. A careful evaluation of the periosteal reaction and appearance of the underlying cortex, in combination with the MRI, clinical, and laboratory data, provides valuable information on lesion duration and aggressiveness, thereby assisting in the etiological diagnosis and optimizing patient management. A solid reaction strongly suggests a benign and slow-growing process that gives the bone enough time to wall off the lesion. Single lamellar reactions occur in acute and usually benign diseases. Multilamellar reactions are associated with intermediate aggressiveness and a growth rate close to the limit of the walling-off capabilities of the bone. Spiculated, interrupted, and complex combined reactions carry the worst prognosis, as they occur in the most aggressive and fast-growing diseases: the periosteum attempts to create new bone but is overwhelmed and may be breached.

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Hair-on-end appearance in a case of thalassemia intermedia

Cited by 8 publications

References 3 publications

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease

Hair-on-End Sign in a 9-Year-Old Girl Presenting with Acute Stroke in Sickle Cell Disease

A new approach for the diagnosis of b-thalassemia in archaeological contexts: the relationship between congenital anaemia and dentinogenesis defects through micro-CT

Periosteum: Characteristic imaging findings with emphasis on radiologic-pathologic comparisons

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