WBC DISORDERS.ppt…………………………………………………………..

General patholgy

1. WBC DISORDERS
LEUKEMIA / LYMPHOMA

2. Leukemia
VS
LEUKEMOID REACTION

7. Leukemia is a clonal malignant
disease of hematopoietic
progenitor cells.
literally means WHITE BLOOD

9. LEUKEMIA
ACUTE
CHRONIC
MYELOID
LYMPHOID

10. PRESENTATION ACUTE CHRONIC
ONSET ABRUPT INSIDIOUS
AGE ALL AGES ADULTS
TOTAL COUNT LOW/NORMAL/
ELEVATED
ELEVATED
BLASTS 20 OR
ABOVE 20 %
BELOW 20
%
ORGANOMEGALY MILD SEVERE

11. ACUTE
LEUKEMIAS
Myeloid
Lymphoid

12. AML ALL
MALIGNANT
PROLIFERATION OF
MYELOID
PROGENITORS
MALIGNANT
PROLIFERATION OF
IMMATURE
LYMPHOCYTES
85 % ABOVE 15 YEARS
( 15 –39 )
85 % BELOW 15 YEARS
(2 –10 )
PHILADELPHIA
CHROMOSOME -VE
PHILADELPHIA
CHROMOSOME + VE

13. ETIOLOGY
MOSTLY A CHROMOSOMAL
ABNORMALITY.
RADIATION EXPOSURE.
CHEMOTHERAPY.
DOWN SYNDROME.
DNA REPAIR GENE MUTATION.

14. MYELOBLAST
LYMPHOBLAST

15. MORPHOLOGY -AML
M 3
Auer rods

17. L 1
L 2
L 3
ALL -FAB

18. MARROW

19. CHRONIC
LEUKEMIAS
MYELOID
LYMPHOID

20. CML CLL/SLL
IT’S ONE OF
MPD.TUMOR OF
PLURIPOTENT
STEM CELL
PROBLEM WITH
LYMPHOID STEM
CELL . ALMOST B
CELL TYPE
25 –60 YRS PEAK –
4
TH
& 5
TH
DECADE
> 50 YRS
PHILADELPHIA
CHROMOSOME +
GENETIC FINDINGS
RARE

21. CLINICAL PROGRESSION
CML

22. CML

23. ETIOLOGY OF CML

24. MORPHOLOGY

25. CML-CHRONIC PHASE

26. GARDEN PARTY APPEARANCE

27. SMEAR -CLL
SMUDGE CELLS
MATURE
LYMPHOCYTES

28. SCHOOL GIRLS APPEARANCE

29. CLINICAL PRESENTATION
What is the main problem with
a malignant lesion?

30. Symptoms are due to
1)Space consumption by the
tumor
2)Tissue infiltration
3)Viscosity of blood
4)Extramedullary hematopoiesis
5)High cell turnover

32. Anaemia

33. Neutropenia with infections
& fever

34. Bleed

35. Due to tissue
infiltration

36. Organomegaly

37. GENERALIZED
LYMPHADENOPATHY

38. Gum hypertrophy

39. INVESTIGATIONS
CBC
PERIPHERAL SMEAR
STUDY
BONEMARROW
-ASPIRATION
-TREPHINE
IMMUNOPHENOTYPING
CYTOGENETICS/
MOLECULAR GENETICS

40. LYMPHOID
NEOPLASMS
HODGKIN AND NONHODGKIN
LYMPHOMA

41. LYMPHOMA
HODGKIN
LYMPHOMA
NONHODGKIN
LYMPHOMA
SINGLE AXIAL GROUP
OF NODES
MULTIPLE PERIPHERAL
NODES
CONTIGUITY OF
SPREAD
NONCONTIGUOUS SPREAD
WALDEYER RING ,
MESENTERIC NODES
RARELY INVOLVED
COMMONLY INVOLVED
EXTRANODAL
INVOLVEMENT RARE
COMMON

42. CLASSIFICATION
1994 REVISED EUROPEAN AMERICAN
CLASSIFICATION OF LYMPHOID
NEOPLASMS (REAL)
WHO –UPDATED REAL
CLASSIFICATION

43. WHO CLASSIFICATION
PRECURSOR B CELL NEOPLASMS
PERIPHERAL B CELL NEOPLASMS
PRECURSOR T CELL NEOPLASMS
PERIPHERAL T/ NK CELL NEOPLASMS
HODGKIN LYMPHOMA

44. B CELL NEOPLASMS

45. T / NK CELL NEOPLASMS

46. HODGKIN LYMPHOMA

49. Hodgkin lymphoma
Only 0.7% of all cancers but disease generally
of young (mean age at dx 32)
Bimodal (second peak in elderly)
??associated with EBV (mixed cellularity 70%
+ vs nodular sclerosing 0%)

50. HISTORY
THOMAS HODGKIN -1832
WILKS -Rediscovered the original article
1865
Reed and Sternberg described tumor giant
cells –Reed-sternberg cells

53. CELL OF ORIGIN
GERMINAL CENTRE OR POST
GERMINAL CENTRE B
CELL

54. EBV ASSOCIATION
FREQUENT PRESENCE OF EBV
EPISOMES IN R S CELLS OF MANY
CASES OF MIXED CELLULARITY TYPE.

55. CLINICAL PRESENTATION
PAINLESS ENLARGEMENT OF
LYMPHNODES
SYSTEMIC SYMPTOMS –FEVER,
NIGHT SWEATS, WEIGHT LOSS
ALCOHOL –PAIN IN NODES
HEPATOSPLENOMEGALY

58. REED-STERNBERG CELLS
15-45 micrometer
Multiple nuclei
Each large inclusion
like nucleolus
nucleolus size-small
lymphocyte

60. Mixed cellularity

61. PROGNOSIS
NODULAR SCLEROSIS EXCELLENT
MIXED CELLULARITY VERY GOOD
LYMPHOCYTE RICH GOOD
LYMPHOCYTE DEPLETED WORST
N L P H D BEST

62. What is this cell ?

63. Cart wheel or clock face appearence

64. Plasma cell
What is it’s function ?

66. What is the
neoplasm that arise
from this cell ?

67. MULTIPLE
MYELOMA
PLASMA CELL MYELOMA

68. MULTIPLE MYELOMA IS A PLASMACELL
NEOPLASM CHARACTERIZED BY
INVOVEMENT OF THE SKELETON AT
MULTIPLE SITES.

69. MULTIPLE MYELOMA
MOST COMMON BONE TUMOR
OLD AGE
M > F
COMMON SITE –BONE
OTHER SITES –LYMPHNODE ,
SKIN

70. CLINICAL PRESENTATION
DEPENDS ON
BONE INVOLVEMENT
INCREASED ABNORMAL Ig
PRODUCTION
DECREASED IMMUNITY
MARROW FAILURE
RENAL FAILURE
AMYLOIDOSIS

74. Î Abnormal Ig & light chain production
IgG–55 % , IgA –25 % ,others rare.
IgA is associated with hyperviscosity.
Light chain –bencejones proteins.
> 3 gms/ dl Igin serum (M protein).
> 6 gms/ dl BJ protein in urine.
60 –70 % both BJ & M proteins +
20 % only BJ protein,
1% nonsecretory.

76. Amyloidosis

78. Etiology &
pathogenesis

79. Environmental Genetic
Radiation exposure
Paper industry
Wood work
Petroleum , benzene
Plastic & rubber
industry
Hyperdiploidy
Trisomy 3,7,9,11
Monosomy13,x
Translocation
involving 14
Deletion of 13q
Rb& RAS
mutation
P53 mutation

82. Factors which help survival &
proliferation of plasma cells
IL –6
TGF –β
IL -1α
IL -1β
VEGF
bFGF
GM -CSF

83. MORPHOLOGY

84. PERIPHERAL SMEAR

87. INVESTIGATIONS

88. X RAY
PROTEIN ELECTROPHORESIS
ESR
CBC
SMEAR & BONEMARROW
IMMUNOPHENOTYPING
URINE BENCE JONES
PROTEIN

90. SULPHOSALICYLIC ACID
TEST
BENCE JONES PROTEIN