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Sclerosing Extramedullary Hematopoietic Tumor: A Case Report
Dapeng Wang1, Eduardo Castro1, Arundhati Rao1
1Baylor & Scott White Health, Temple, TX, USA.
Journal of Investigative Medicine High Impact Case Reports
|September 11, 2020
Summary
Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare condition often seen in patients with myeloproliferative disorders. This case highlights SEMHT in a patient with acute lymphoblastic leukemia, emphasizing diagnostic considerations for intra-abdominal masses.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Sclerosing extramedullary hematopoietic tumor (SEMHT), previously known as fibrous hematopoietic tumor or myelosclerosis, is a rare extramedullary proliferation of hematopoietic tissue.
- It typically occurs in patients with a history of chronic myeloproliferative disorders and can affect various organs, including the liver.
- Diagnosis requires careful evaluation of tissue biopsy, especially in patients with a known hematological disorder.
Observation:
- A 37-year-old female with a 5-year history of acute B-cell lymphoblastic leukemia presented with right upper quadrant pain.
- CT scans revealed multiple ring-enhancing liver lesions.
- A liver biopsy showed extramedullary hematopoiesis with atypical megakaryocytes, sinusoidal capillarization, and fibroblastic proliferation, consistent with SEMHT.
Findings:
- Bone marrow examination revealed extensive reticulin fibrosis (MF-3), trilineage dysplasia, increased blasts (10-19%), and hypercellularity, indicative of myelodysplastic syndrome with excess blasts, type 2 (MDS-EB-2).
- Cytogenetic analysis showed complex abnormalities, including chromosome 7 deletion.
- Molecular testing was negative for JAK2 V617F and CALR exon 9 mutations.
Implications:
- This case contributes a rare instance of SEMHT diagnosed synchronously with MDS-EB-2 and significant chromosomal abnormalities.
- The findings underscore the importance of considering SEMHT in the differential diagnosis of intra-abdominal masses in patients with hematological disorders.
- The poor clinical evolution associated with chromosome 7 deletion highlights the need for vigilant management and further research into SEMHT pathogenesis and treatment.
