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CASE 14
ā€¢ 54 year male
ā€¢ Complaints of low back pain- 6 mths
ā€¢ USG: presacral mass
ā€¢ Guided FNAC was performed
DIAGNOSIS
ā€¢ History of repeated blood transfusions:
weakness, fatigue- since 6 years
ā€¢ CT scan: gross splenomegaly ;
Retroperitoneal sarcoma
ā€¢ BONE MARROW ASPIRATION: hypercellular
marrow (outside)
ā€¢ BMA (KMIO) : hypocellular aspirate
ā€¢ BMB : predominantly adipocytes with focus of
marrow fibrosis
ā€¢ HEMOGLOBIN ELECTROPHORESIS
HbF ā€“ 5.70%
HbA ā€“ 78.4%
HbA2 ā€“ 2.20%
Others
Cell Block Study
Response
ā€¢ Malignant carcinoma
ā€¢ Chordoma
ā€¢ Malignant tumour
EXTRAMEDULLARY
HEMATOPOIESIS
EXTRAMEDULLARY HEMATOPOIESIS
ā€¢ DEFINITION: Hematopoiesis occurring in
organs outside of the bone marrow.
ā€¢ It occurs in diverse conditions, including fetal
development, normal immune responses, and
pathological circumstances.( HEMATOLOGICAL
AND NON HEMATOLOGICAL )
Compensatory process
ā€¢ Occur whenever and wherever a stem cell
niche/microenvironment is present in a tissue
ā€¢ Sites: adults are most commonly liver, spleen,
and lymph nodes.
ā€¢ Rarely, central nervous system.
Theories
ā€¢ Compensatory phenomenon with increased
numbers of circulating hematopoietic stem
cells.(marrow failure)
ā€¢ Myelostimulatory theory, which proposes that
EMH presents in fetal hematopoietic sites
under stimulation of unknown
myelostimulatory factors
ā€¢ ā€œRedirected differentiation theory.ā€
ā€¢ Presumed bone marrow embolism
ā€¢ Choreography of stromal, humoral and stem
cell interactions is a 3-dimensional interplay
that relies on much more than physical space.
ā€¢ Although trilineage; 1 or 2 cell types may
predominate.
ā€¢ ā€œExtramedullary hematopoiesisā€ refers to a
broad range of hematopoietic activities from
the early stages of lineage commitment to late
stages of hematopoietic cell maturation.
ā€¢ Does not fully supplant the marrow
hematopoiesis, but imperative for the
maturation of hematopoietic cells.
Case 14
Case 14
Case 14

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Case 14

  • 1.
  • 3. ā€¢ 54 year male ā€¢ Complaints of low back pain- 6 mths ā€¢ USG: presacral mass ā€¢ Guided FNAC was performed
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 9.
  • 11. ā€¢ History of repeated blood transfusions: weakness, fatigue- since 6 years ā€¢ CT scan: gross splenomegaly ; Retroperitoneal sarcoma
  • 12.
  • 13. ā€¢ BONE MARROW ASPIRATION: hypercellular marrow (outside) ā€¢ BMA (KMIO) : hypocellular aspirate ā€¢ BMB : predominantly adipocytes with focus of marrow fibrosis ā€¢ HEMOGLOBIN ELECTROPHORESIS HbF ā€“ 5.70% HbA ā€“ 78.4% HbA2 ā€“ 2.20% Others
  • 15.
  • 16. Response ā€¢ Malignant carcinoma ā€¢ Chordoma ā€¢ Malignant tumour
  • 18. EXTRAMEDULLARY HEMATOPOIESIS ā€¢ DEFINITION: Hematopoiesis occurring in organs outside of the bone marrow. ā€¢ It occurs in diverse conditions, including fetal development, normal immune responses, and pathological circumstances.( HEMATOLOGICAL AND NON HEMATOLOGICAL )
  • 19. Compensatory process ā€¢ Occur whenever and wherever a stem cell niche/microenvironment is present in a tissue ā€¢ Sites: adults are most commonly liver, spleen, and lymph nodes. ā€¢ Rarely, central nervous system.
  • 20. Theories ā€¢ Compensatory phenomenon with increased numbers of circulating hematopoietic stem cells.(marrow failure) ā€¢ Myelostimulatory theory, which proposes that EMH presents in fetal hematopoietic sites under stimulation of unknown myelostimulatory factors ā€¢ ā€œRedirected differentiation theory.ā€ ā€¢ Presumed bone marrow embolism
  • 21. ā€¢ Choreography of stromal, humoral and stem cell interactions is a 3-dimensional interplay that relies on much more than physical space. ā€¢ Although trilineage; 1 or 2 cell types may predominate.
  • 22. ā€¢ ā€œExtramedullary hematopoiesisā€ refers to a broad range of hematopoietic activities from the early stages of lineage commitment to late stages of hematopoietic cell maturation. ā€¢ Does not fully supplant the marrow hematopoiesis, but imperative for the maturation of hematopoietic cells.