Rbc disorders 8

3,737 views

Published on

Target: UG medical students.

Published in: Health & Medicine
1 Comment
9 Likes
Statistics
Notes
No Downloads
Views
Total views
3,737
On SlideShare
0
From Embeds
0
Number of Embeds
2
Actions
Shares
0
Downloads
198
Comments
1
Likes
9
Embeds 0
No embeds

No notes for slide

Rbc disorders 8

  1. 1. Aplastic AnemiaDr.CSBR.Prasad, M.D.,
  2. 2. Definition• Aplastic anemia refers to a syndrome of chronic primary hematopoietic failure and attendant pancytopenia – Anemia – Neutropenia, and – Thrombocytopenia
  3. 3. MechanismsStem cell defect:• Autoimmune (Most common)• Inherited or• Acquired
  4. 4. Major Causes of Aplastic Anemia
  5. 5. Aplastic AnemiasFanconi anemia• AR disorder• Defect in DNA repair• Marrow hypoplasia• Hypoplasia of kidney, spleen• Hypoplastic anomalies of bone – Thumb, Radius
  6. 6. Fanconi anemia
  7. 7. Aplastic Anemias - PathogenesisEnvironmental factors trigger the production of genetically altered stem cells1. Immunologically mediated suppression of stem cells By cytokines released by T cells2. Intrinsic abnormality of stem cells ↓ proliferation & differentiating capacity
  8. 8. Pathophysiology of aplastic anemia
  9. 9. Aplastic Anemias - Morphology• BM Aspiration – Dry tap• BM biopsy – Diagnostic• BM – markedly hypocellular• Empty marrow spaces - fat, fibrous tissue, lymphocytes, plasma cells
  10. 10. Aplastic anemia
  11. 11. Morphology
  12. 12. Aplastic AnemiasPBS• NCNC, slight macrocytosis• Reticulocytosis is absent• Leukopenia• Thrombocytopenia
  13. 13. Clinical Features• Can occur at any age• Onset is usually insidious• Initial manifestations vary depending on the affected cell line• Splenomegaly is absent• Reticulocytopenia is the rule If it is present, the diagnosis of aplastic anemia should be seriously questioned
  14. 14. Aplastic Anemia• DD – MDS – Aleukemic leukemiaTreatment• Allogenic BMT• Antithymocyte globulin along with cyclosporine
  15. 15. Mary Curie
  16. 16. Pure Red Cell Aplasia
  17. 17. • It’s a primary marrow disorder in which only erythroid progenitors are suppressed• Causes: – Thymoma – Large granular lymphocytic leukemia – Drug exposures – Autoimmune disorders – Parvovirus infection B19
  18. 18. Myelophthisic anemia
  19. 19. Myelophthisic anemiaSOL destroys BM architecture & depresses its productive capacityCauses1. Metastatic cancer – lung, breast, prostate, thyroid2. Multiple myeloma, leukemia, lymphoma3. MyelofibrosisPBS - Leukoerythroblastosis
  20. 20. Polycythemia
  21. 21. Polycythemia• Myeloproliferative disorder – Unregulated proliferation of erythroid elements in BM – ↑ Erythrocyte concentration in peripheral blood• Also ↑ other blood cells• Synonyms: Polycythemia rubra vera, primary polycythemia, erythemia
  22. 22. Polycythemia• Polycythemia Vera – primary• Secondary Polycythemia – Appropriate to erythropoietin production • High altitude • COPD • Obesity – Inappropriate to erythropoietin production • Tumors – RCC, HCC, uterine leiomyoma • Renal ischemia – Familial Polycythemia• Relative Polycythemia – Gaisbock’s syndrome – Dehydration
  23. 23. Polycythemia Vera• Unknown cause• Possible mechanisms – Erythropoietin independent proliferation of neoplastic stem cells – Hypersensitivity of erythroid stem cells to erythropoietin – Hypersensitivity of erythroid stem cells to insulin growth factors – Abnormal GF that act on normal stem cells
  24. 24. Polycythemia Vera – C/F• 40- 60 years• M>F• Gradual in onset• Headache, weakness, pruritis, weight loss, fatigue• GIT bleeding, menorrhagia, hemoptysis• MI, Renal vein thrombosis, cerebral ischemia
  25. 25. Plethora of polycythemia
  26. 26. Polycythemia Vera – C/F• Splenomegaly – 75%• Hepatomegaly – 50%• Hypertension – 50%• Long standing cases – myelofibrosis – 30%• 10% cases develop into acute leukemia
  27. 27. Polycythemia Vera – Lab findings• Absolute Erythrocytosis: > 6-10millions/cumm• Hemoglobin: > 18 gm%• PCV > 48% in F, > 52% in M• ESR – 2-3mm/ hour• Reticulocyte count – normal / mildly increased• Leukocytosis: > 12,000/mm3• Platelet count - ↑• PBS – NNBP, MCHC
  28. 28. Polycythemia Vera – Lab findings• BM – – hypercellular – 95% cases - ↑ megakaryocytes – ↑ reticulin• Treatment – Phlebotomy – Myelosuppresive therapy
  29. 29. END
  30. 30. Dr.CSBR.Prasad, M.D.,Associate Professor of Pathology,Sri Devaraj Urs Medical College, Kolar-563101, Karnataka, INDIA. csbrprasad@reiffmail.com

×