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Case Reports
. 2022 Mar 25:23:e935141.
doi: 10.12659/AJCR.935141.

A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome

Krzysztof Gawroński  1 , Kamila Kruczkowska-Tarantowicz  1 , Piotr Rzepecki  1 , Daniel Lisicki  2
Affiliations
Case Reports

A Case of Multifocal Extramedullary and Non-Hepatosplenic Extramedullary Hematopoiesis in a 43-Year-Old Man with a History of Congenital Eisenmenger Syndrome

Krzysztof Gawroński et al. Am J Case Rep. .

Abstract

BACKGROUND Extramedullary hematopoiesis in organs outside the bone marrow most commonly occurs in the liver and spleen. This report is of a case of multifocal extramedullary and non-hepatosplenic extramedullary hematopoiesis a 43-year-old man with a history of congenital Eisenmenger syndrome. CASE REPORT We present the case of a 43-year-old patient with complex heart disease and full-blown Eisenmenger syndrome associated with ventricular septal defect, bicuspid right ventricle, and pulmonary hypertension. In July 2020, the patient began to report neurological symptoms in the form of lower-limb numbness and weakness of lower-limb strength, with additional increasing lower-limb edema. A CT scan of the abdomen and pelvis revealed a 63×102×103 mm soft-tissue mass in the pelvis, located behind the urinary bladder. Due to the suspicion of proliferative disease, mainly of the lymphatic system, a diagnostic trephine biopsy was performed first, but no tumor cell infiltration was found. Then, the patient was qualified for diagnostic surgery. During the operation, tumor sections were harvested. Histopathological examination of the tissue sections showed extramedullary hematopoiesis in the tumor lining. Hydroxycarbamide was used as first-line treatment. However, it was not effective in controlling clinical symptoms. Therefore, the patient was qualified for radiotherapy as a second-line palliative treatment. CONCLUSIONS This report presents a patient with cyanotic heart disease and extramedullary and non-hepatosplenic hematopoiesis presenting as masses that mimicked malignancy. In this case, palliative radiotherapy effectively reduced the symptoms due to the size of the mass lesion.

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Conflict of interest statement

Conflict of interest: None declared

Figures

Figure 1.
Figure 1.
A photomicrograph of the histopathology of the mass showing extramedullary hematopoiesis with blast cells of all hematologic cell lines.
Figure 2.
Figure 2.
(A) Myeloperoxidase. (B) CD71 immunochemistry stains. (C) Sparse CD34-positive blasts. (D) Increased number of CD117-positive cells.
Figure 3.
Figure 3.
CT scan with abnormal masses (arrows indicate pathological masses).
See this image and copyright information in PMC

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References

    1. Short C, Lim H, Tan JKH, O’Neill HC. Targeting the spleen as an alternative site for hematopoiesis. BioEssays. 2019;41(5):e1800234. - PubMed
    1. Sohawon D, Lau KK, Lau T, Bowden DK. Extra-medullary haematopoiesis: A pictorial review of its typical and atypical locations. J Med Imaging Radiat Oncol. 2012;56(5):538–44. - PubMed
    1. Katchi T, Kolandaivel K, Khattar P, et al. Extramedullary hematopoiesis presented as cytopenia and massive para spinal masses leading to cord compression in a patient with hereditary persistence of fetal hemoglobin. Biomark Res. 2016;4(1):17. - PMC - PubMed
    1. Taylor CL, Maynard F, Liebman J, et al. Extramedullary hematopoiesis causing paraparesis in congenital cyanotic heart disease. Neurology. 1998;51(2):636–37. - PubMed
    1. Karimi M, Cohan N, Pishdad P. Hydroxyurea as a first-line treatment of extramedullary hematopoiesis in patients with beta thalassemia: Four case reports. Hematology. 2015;20(1):53–57. - PubMed

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