Case 14
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A 54-year-old male presented with 6 months of low back pain. Imaging found a presacral mass and bone marrow biopsy showed hypocellular marrow with focus of marrow fibrosis. Hemoglobin electrophoresis showed elevated HbF. Cell block study was positive for malignant carcinoma. The patient had a history of repeated blood transfusions and anemia for 6 years. Further reading explained that extramedullary hematopoiesis is the occurrence of hematopoiesis outside the bone marrow, which can be a compensatory process in conditions that reduce bone marrow function, such as bone marrow failure or infiltration.
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Case 14
- 2. CASE 14
- 3. • 54 year male • Complaints of low back pain- 6 mths • USG: presacral mass • Guided FNAC was performed
- 10. DIAGNOSIS
- 11. • History of repeated blood transfusions: weakness, fatigue- since 6 years • CT scan: gross splenomegaly ; Retroperitoneal sarcoma
- 13. • BONE MARROW ASPIRATION: hypercellular marrow (outside) • BMA (KMIO) : hypocellular aspirate • BMB : predominantly adipocytes with focus of marrow fibrosis • HEMOGLOBIN ELECTROPHORESIS HbF – 5.70% HbA – 78.4% HbA2 – 2.20% Others
- 14. Cell Block Study
- 16. Response • Malignant carcinoma • Chordoma • Malignant tumour
- 18. EXTRAMEDULLARY HEMATOPOIESIS • DEFINITION: Hematopoiesis occurring in organs outside of the bone marrow. • It occurs in diverse conditions, including fetal development, normal immune responses, and pathological circumstances.( HEMATOLOGICAL AND NON HEMATOLOGICAL )
- 19. Compensatory process • Occur whenever and wherever a stem cell niche/microenvironment is present in a tissue • Sites: adults are most commonly liver, spleen, and lymph nodes. • Rarely, central nervous system.
- 20. Theories • Compensatory phenomenon with increased numbers of circulating hematopoietic stem cells.(marrow failure) • Myelostimulatory theory, which proposes that EMH presents in fetal hematopoietic sites under stimulation of unknown myelostimulatory factors • “Redirected differentiation theory.” • Presumed bone marrow embolism
- 21. • Choreography of stromal, humoral and stem cell interactions is a 3-dimensional interplay that relies on much more than physical space. • Although trilineage; 1 or 2 cell types may predominate.
- 22. • “Extramedullary hematopoiesis” refers to a broad range of hematopoietic activities from the early stages of lineage commitment to late stages of hematopoietic cell maturation. • Does not fully supplant the marrow hematopoiesis, but imperative for the maturation of hematopoietic cells.