White blood cell disorders

1
White blood cell disorders

• Leukopenia
• Neutrophenia
• Lymphocytopenia
• Leukocytosis
• Granulocytosis
• Lymphocytosis
• Neoplastic proliferations of white cells
• Malignant lymphoma
• Leukemias

2
Neutropenia

• neutrophil?
• lt 1,500 cells/ml (????)
• lt 1,800 cells/ml (???????)
• agranulocytosis (lt500 cells/ml)
• ?????????????????? 6-7 hr
• ????????????????? ?????????? ????????????
• ?????? ???????????????? ????????? ???????
  (???????? 16 ???? 38) ??????????? ???????????????
  SLE , ??????????? neutrophil ?????????????????????
  ????????

3
Neutrophilia

• Neutrophil gt10,000 cells/ml
• ??????????? leukemoid reaction (50,000
  cells/ml)
• ??????? leukemia ???????? immature cells ????????
• ??????????????????? ???? ??????????????????????
• ?????? ???????? 17 ???? 39
• ???????? ?????????????????? ??????
  glucocorticoids, immunosuppressants

4
Eosinopenia

• eosinophillt 50 cells/ml ?????????????
• ?????? ????????????????? ??? epinephrine,
  glucocorticoids

5
Eosinophilia

• ???????? 3 ?????
• mild gt 400-1500 cells/ml
• moderate gt 1500-5000 cells/ml
• marked (high) gt 5000 cells/ml
• ?????? ????????????????? ?????? ?????????? ?
• ???????? 18 ???? 40

6
Basophilopenia

• ??????? 20-80 cells/ml
• ?????????? 10 cells/ml ?????????????? ??? diag.
  ???????????????????????????
• ??????
• ????? glucocorticoids ????? ???? ???????????
  glucocorticoids ????
• hyperthyroidism
• ovulation
• hypersensitivity reaction

7
Basophilia

• gt 300 cells/ml
• ??????
• ?????? ?????? ulcerative colitis
• ?? ??????? ?????????
• hypothyroidism (myxedema)
• infection ????? ????? ??????
• ???????? ?????????????
• mastocytosis urticaria pigmentosa

8
Monocytopenia

• lt 200 cells/ml
• ???????????????????????????????????? ?
• ???? aplastic anemia, leukemia, SLE, RA
• ????????? IFN-alpha, glucocorticoids, TNF-alpha

9
Monocytosis

• gt 800 cells/ml
• ???????????????????????????????????? ?
• ???? AML, CML, lymphoma, myeloma, SLE, RA,
  alcoholic liver disease, tetrachloroethane
  poisoning

10
Lymphocytopenia

• lymphocyte ?
• lt 3,000 cells/ml (adult)
• lt 1,500 cells/ml (child)
• ?????? stress, AIDS, corticosteroid therapy,
  radiation, toxic drugs, Cushings syndrome

11
Lymphocytosis

• Lymphocyte?
• gt 4,000 cells/ml (adult)
• gt 9,000 cells/ml (child)
• ??????
• ???????????????????? adrenal corticosteroid
  hormones ????
• ????????????? (common)

12
Neoplastic proliferations of white cells

• Lymphoma (???????????????????)
• lymphoid tissues (solid tumor)
• Non-Hodgkins lymphomas (NHLs)
• Hodgkins lymphoma
• ???????? 19 ???? 42

• Leukemias (??????????????????)
• ??????????? myeloid, lymphoid cell acute,
  chronic
• Acute lymphocytic leukemia (ALL) Acute
  myelocytic leukemia (AML)
• CLL, CML

13
Acute Leukemias (FAB)

• ALL
• 3 subgroups L1-L3
• L1 (children)
• L2 (older children, adults)
• L3 (secondary to lymphoma)
• WHO ????????
• T cell ALL
• B cell ALL

• AML
• 7 subgroups M1-M7
• M1 (myeloblasts without differentiation)
• M2 (myeloblasts with some diff.)
• M3 (acute promyelocytic)
• M4 (acute myelomonocytic)
• M5 (acute monocytic)
• M6 (erythroleukemia)
• M7 (megakaryoblastic)
• (???????? 20 ???? 43)

14
Acute leukemias

• Blasts ? ?? marrow ????????????????????????????
• ?????
• anemia
• ???
• ????????????
• ????????
• ???????????????
• ?????????
• ?????????
• ????????????? CNS

15
Chronic Leukemias

• CML
• ? neutrophil
• adult 40-50 yrs.
• ?????????????? ????????????? 3 ?? ? acute
  leukemia (blast crisis)

• CLL
• B lymphocyte
• adult 50 yrs. ?????????????? ?????????
  ?????????? ????????? ????????????? ??? ??????

16
Treatment

• Supportive treatment
• Chemotherapy
• AML anthracycline (duanorubicin) cyt
  arabinosine (6-thioguanine)
• ALL vincristine, prednisolone, L-asperaginase
• CML hydroxyurea, busulfan, alpha interferon
• CLL chlorambucil, CHOP (cyclophosphamide
  doxorubicin vincristine prednisolone)
• Stem cell transplantation

17
Platelet disorders

• Quantitative disorders
• thrombocytopenia (40,000/ mL)
• thrombocytosis (1,000,000/ mL )

• Qualitative disorders
• Inherited ???? VWD
• Acquired ???? chronic liver disease

18
Coagulation disorders

• Hemophilias
• Coagulation disorders
• Vitamin K deficiency
• DIC
• Afrinogenemia

19
Pro-and anticoagulant activities of endothelial
cells
Mitchell, RN, and Cotran, RS, p.86, 2003
20
Coagulation cascade
http//www.life.sci.qut.edu.au/dow/olmt/haemostasi
s/coagulation3.htm
21
Coagulation Factors

• Factor II, VII, IX, X Vit K dependent factors
• Factor XI, XII, prekallikrein HMW kininogen
  contact activation factors
• Factor V, VIII, XIII, fibrinogen
  thrombin-sensitive factors
• ???????? 21 ???? 46

22
Platelet and Coagulation disorders

• Essential thrombocythemia
• hemophilia A, B
• von Willebrand disease (VWD)
• Vitamin K deficiency
• Liver disease
• ???????? 22 ???? 48 (Lab)

23
Essential thrombocythemia (ET)

• Myeloproliferative disorder ????????????
  polcythemia vera
• LAB
• Platelet gt 600,000 /?L
• Leukocytosis, erythrocytosis
• Megakaryocytic hyperplasia (bone marrow)
• ????? ??????? ?????? 50-70 ??
• ??????? 2 episodes hemorrhage thrombosis
• ?????????????? ???????? ??? ???????????? ???????
  thrombosis
• ???????????? ????????????? aspirin
• thrombosis ??????? artery ???? coronary
• ???????? ??? hydroxyurea, IFN-gamma
• follow up Plt count
• Transformation to AML, polcythemia vera

24
Hemophilia A, B

• X-linked recessive disorder
• A factor VIII, B factor IX
• ????? ????? hemorthroses ????????????
  ??????????????? ?????????????????????????? factor
• ??? aPTT ???????
• ?????????? Lab (???????? 24 ???? 50)

25
Hemophilia A, B (Cont)

• ????????
• ??????????????????????????? bleeding
• ??????????????? bleeding complications
• replacement with factor
• Desmopressin ?????????? DDAVP ????????? mild
  hemophilia A
• antifibrinolytic agents ???? epsilon-aminocaproic
  acid, tranexamic acid ????? hemophilia A

26
Von Willebrand disease (VWD)

• ????????????
• vWF subunit from endothelial cells ?mutimer
  ??????????????? HMW
• vWF ?????????
• ?????? Plt-subendothelium
• bind and stabilize Factor VIII
• 3 types (I, II (2A, 2B, 2M, 2N), III)
• ????????????
• Lab BT ???, Ptl ????, aPTT ??????????
• ?????????
• vWFAg
• vWFRco (ristocetin) ???? HMW
• Factor VIII acitivity ??????????????

27
Vitamin K Deficiency

• Newborn hemorragic disease
• Vit K - dependent factor ???, PT, aPTT, TT
  ???????
• Phytonadione (K1) IM, as soon as deliver

28
Liver disease

• Liver ?????????? coagulation factors
• II, VII, IX, X, XII, V, fibrinogen
• inhibitor anti-thrombin, protein C, S
• ??????????????????????????
• ?????????????
• factor replacement

29
References

• ????? ?????????????? (2545) ???????????????????
  ?? ????????? ???????????????, ????? ???????????,
  ???????? ???????????, ???? ?????? ??? ?????
  ????????? (??????????) ????????????????,
  ???????????????????????? (????), ?????????????
  ???? 320-325.
• 2. ????? ?????????????? (2545) ???????????????????
  ???????????? ?? ????????? ???????????????, ?????
  ???????????, ???????? ???????????, ???? ??????
  ??? ????? ????????? (??????????)
  ????????????????, ????????????????????????
  (????), ????????????? ???? 331-338.
• 3. ?????? ????????? (2545) ???????????????????????
  ????????????????????? ?? ?????????
  ???????????????, ????? ???????????, ????????
  ???????????, ???? ?????? ??? ????? ?????????
  (??????????) ????????????????, ???????????????????
  ????? (????), ????????????? ???? 243-258.
• 4. ?????? ????????? (2545) ???????????????????????
  ????????????????????????? ?? ?????????
  ???????????????, ????? ???????????, ????????
  ???????????, ???? ?????? ??? ????? ?????????
  (??????????) ????????????????, ???????????????????
  ????? (????), ????????????? ???? 272-286.
• 5. ???? ?????????? (2545) ????????????????????????
  ?? ????????? ???????????????, ????? ???????????,
  ???????? ???????????, ???? ?????? ??? ?????
  ????????? (??????????) ????????????????,
  ???????????????????????? (????), ?????????????
  ???? 235-242.
• 6. ?????? ???????????? (2545) ???????????????????
  ?? ????????? ???????????????, ????? ???????????,
  ???????? ???????????, ???? ?????? ??? ?????
  ????????? (??????????) ????????????????,
  ???????????????????????? (????), ?????????????
  ???? 371-386.
• 7. ?????? ??????????? (2545) ?????????????????????
  ?????????????? ?? ????????? ???????????????,
  ????? ???????????, ???????? ???????????, ????
  ?????? ??? ????? ????????? (??????????)
  ????????????????, ????????????????????????
  (????), ????????????? ???? 287-295.
• 8. ?????? ???????????? (2545) ????????????????????
  ?????? ?? ????????? ???????????????, ?????
  ???????????, ???????? ???????????, ???? ??????
  ??? ????? ????????? (??????????)
  ????????????????, ????????????????????????
  (????), ????????????? ???? 259-271.

30
References

• 9. ?????? ???????????? (2545) ????????????????????
  ?????????????? ?? ????????? ???????????????,
  ????? ???????????, ???????? ???????????, ????
  ?????? ??? ????? ????????? (??????????)
  ????????????????, ????????????????????????
  (????), ????????????? ???? 296-309.
• 10. ?????? ???????????? (2545) ???????????????????
  ????????????????? ?? ????????? ???????????????,
  ????? ???????????, ???????? ???????????, ????
  ?????? ??? ????? ????????? (??????????)
  ????????????????, ????????????????????????
  (????), ????????????? ???? 310-319.
• 11. Aster, J. (2003) The hematopoietic and
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• 12. Bickert, B., and Kwaitkowski, J.L. (2002)
  Coagulation disorders in DiPiro, J.T., Talbert,
  R.L., Yee, G.C., Matzke, G.R., Wells, B.G. and
  Posey, L.M. (eds.) Pharmacotherapy A
  Pathophysiologic Approach, 5th ed., McGraw Hill,
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• 13. Curry,Jr., C.E., and Beasley, E.D. (2002)
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31
References

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