Adrenal extramedullary hematopoiesis associated with beta-thalassemia trait in an adult woman: a case report and review of literature

Al-Thani, Hassan; Al-Sulaiti, Maryam; El-Mabrok, Gamela; Tabeb, AbdelHakim; El‐Menyar, Ayman

doi:10.1016/j.ijscr.2016.05.024

Cited by 8 publications

(8 citation statements)

References 27 publications

(58 reference statements)

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“…The apparent hematopoiesis that we observe in the fetal adrenal gland is consistent with the fact that the adrenal gland, along with many other organs (e.g., spleen, liver, and lymph nodes), can serve as a site of extramedullary hematopoiesis in adults with pathologic conditions that lead to an increased demand for blood cell production, particularly hemoglobinopathies (185,186). Although occasional islands of extramedullary hematopoiesis have been seen in the adrenal glands of human embryos (126,127), our findings in both the human and mouse provide quantitative evidence that the adrenal gland serves as a normal, albeit minor, site of erythropoiesis during a developmental window that overlaps with the transition of hematopoiesis from the liver to the marrow.…”

Section: Discussionsupporting

confidence: 84%

A human cell atlas of fetal gene expression

Cao

O’Day

Pliner

et al. 2020

Science

489

544

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The gene expression program underlying the specification of human cell types is of fundamental interest. We generated human cell atlases of gene expression and chromatin accessibility in fetal tissues. For gene expression, we applied three-level combinatorial indexing to >110 samples representing 15 organs, ultimately profiling ~4 million single cells. We leveraged the literature and other atlases to identify and annotate hundreds of cell types and subtypes, both within and across tissues. Our analyses focused on organ-specific specializations of broadly distributed cell types (such as blood, endothelial, and epithelial), sites of fetal erythropoiesis (which notably included the adrenal gland), and integration with mouse developmental atlases (such as conserved specification of blood cells). These data represent a rich resource for the exploration of in vivo human gene expression in diverse tissues and cell types.

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Section: Discussionsupporting

confidence: 84%

A human cell atlas of fetal gene expression

Cao

O’Day

Pliner

et al. 2020

Science

489

544

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“…In our case, EMH involved the right adrenal gland. It is interesting that our literature review on EMH also reported right-side predilection, where 13 out of 17 cases of EMH involved the right adrenal gland, 8,[11][12][13][14][15]17,[25][26][27][28][29][30] two cases involved the left adrenal gland 6,9 and two cases involved bilateral adrenal glands. 16,31 A rather striking right-sided predominance was noted in a study done by Kenney et al, where 53 of 72 adrenal myelolipomas involved the right adrenal glands.…”

Section: Discussionmentioning

confidence: 99%

Rare Presentation of Right Adrenal Mass: Extramedullary Haematopoiesis in a Patient with Thalassaemia Intermedia

Wong

Yong

Gan

et al. 2021

JAFES

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Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass.This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.

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“…EMH can be seen in hematologic diseases such as hemoglobinopathies, myeloproliferative disorders, lymphomas, leukemia, and hemolytic anemias (4,5). EMH commonly occurs outside of the bone marrow in the reticuloendothelial system such as the spleen and liver, but can rarely be seen in organs such as brain, adrenals, pleura, bowel, and breast (7)(8)(9)(10). EMH in the adrenals is rare and is thought to be a compensatory mechanism secondary to altered hematopoiesis in patients with hemolytic disorders (8).…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Rapidly Enlarging Myelolipoma in Sickle Cell Disease

Davari

Lee

2020

AACE Clinical Case Reports

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Objective: Adrenal myelolipoma (AM) is a benign tumor composed of mature fat cells and hemopoietic elements. Most AMs are incidental findings on imaging and clinically asymptomatic. The purpose of this case report is to describe a rare case of AM and explore its clinical manifestations, imaging features, and treatment. Methods: In this study, we report a case of a rapidly growing right AM in a patient with uncontrolled hemoglobin sickle cell disease. A 38-year-old male presented to our institution's endocrine surgery clinic for evaluation of an enlarging right adrenal mass. This mass was incidentally found during an abdominal ultrasound performed for transaminitis and thrombocytopenia. Patient was asymptomatic without any abdominal discomfort, back pain, nausea, or vomiting. Results: Patient was lost to follow up until 2018. Follow-up computed tomography scan in 2018 showed the right adrenal mass measuring 12.3 cm in greatest dimension with significant macroscopic fat. Given the imaging features, AM was the presumed diagnosis. However, with a medical history of uncontrolled sickle cell disease, extra-medullary hematopoiesis and rapidly growing liposarcoma could not be ruled out. Surgical excision was performed due to size and significant tumor growth. Diagnosis was confirmed with histopathology and revealed myelolipoma. Conclusion: Image characteristics can be helpful in diagnosis of AM; however, the appearance of this lesion on computed tomography can be similar to other adrenal gland pathologies such as liposarcoma and mass-forming extramedullary hematopoiesis. Percutaneous needle biopsy may be indicated if the diagnosis remains unclear.

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Adrenal extramedullary hematopoiesis associated with beta-thalassemia trait in an adult woman: a case report and review of literature

Abstract: HighlightsAdrenal myelolipoma with extramedullary hematopoiesis (EMH) is a rare condition.EMH could be presented with several clinical hematological disorders.Surgical management becomes inevitable in certain adrenal EMH cases especially in the presence of large adrenal mass.

Cited by 8 publications

References 27 publications

A human cell atlas of fetal gene expression

A human cell atlas of fetal gene expression

Rare Presentation of Right Adrenal Mass: Extramedullary Haematopoiesis in a Patient with Thalassaemia Intermedia

A Rare Case of Rapidly Enlarging Myelolipoma in Sickle Cell Disease

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