qualitative disorders of WBC

1. QUALITATIVE DISORDERS OF WBC
•Presenter : Nabin Chaudhary

2. CONTENTS
• Lazy leukocyte syndrome
• Chediak higashi syndrome
• Infectious mononucleosis
• Leukemia
• Lymphoma

3. LAZY LEUKOCYTE SYNDROME
(SCHWACHMAN SYNDROME)
Defect in neutrophil chemotaxis and deficient random mobility of
neutrophils
Blood Neutrophils cannot migrate at the site of tissue injury
Phagocytic and Bactericidal activities are normal
3

4. • In vivo study demonstrated that the abnormalities in migration are
intrinsic to the granulocyte
• Alteration in the structure or function of micro-filamentous proteinof
the granulocyte membrane
• Disorder of membrane leading to altered deformability
• Disordered function of the micro filamentous protein of the cell
membrane
• Excessive or undue rigidity of neutrophils
• Impaired mobility
• Failure of egress from the bone marrow

5. CLINICAL FEATURES
AGE
Complications occur at the age of 1-2 years due to infections
SYMPTOMS
Most common: Stomatitis, Otitis media and Bronchitis
RECURRENT INFECTIONS
High Chance of Recurrent Infections
5

6. ORAL MANIFESTATIONS
STOMATITIS (Most Common)
PERIODONTITIS
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7. DIAGNOSIS
CLINICAL DIAGNOSIS
• Recurrent Infection, Periodontitis and Stomatitis
LABORATORY DIAGNOSIS
• TLC slightly low
• ANC as low as 100-200 cells/mm3
• Bone Marrow contains normal number of mature neutrophils
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8. MANAGEMENT
ANTIBIOTICS GIVEN TO CONTROL THE
INFECTION
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9. CHÉDIAK-HIGASHI SYNDROME
(BÉGUEZ CÉSAR SYNDROME, CHÉDIAK-STEINBRINCK-HIGASHI SYNDROME)
Described by Béguez Cesar (1943), Steinbrinck (1948), Chédiak (1952) and
Higashi (1954)
Congenital Autosomal Recessive Immunodeficient defect of Granulocytes and
Melanocytes
Abnormal granules are seen in all blood granulocytes resulting in decreased
chemotactic and bactericidal activity.
The condition usually results in death of childhood before the age of 10
Characterized by abnormal intracellular protein transport
LYST or CHS1 gene
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10. CLINICAL FEATURES
Affects all races
Usually appears soon after birth or in children <5 years
Characterized by immune deficient state
Characteristic Clinical Feature:
Partial Oculocutaneous Albinism
(Silvery Hair Syndrome)
Recurrent Infections of Respiratory tract and Sinuses
Easy bruisability and bleeding
Gastrointestinal disturbances
Lymph Node enlargement (Cervical)
May be associated with Malignant Lymphoma
Progressive Neurological Dysfunction
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11. SILVERY HAIR SYNDROME:Defect melanization of
melanosomes i.e. autophagocytosis of melanosomes
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12. ORAL MANIFESTATIONS
GILLIG AND CALDWELL
Ulcerations of the Oral Mucosa
Severe Gingivitis and
Glossitis
HAMILTON AND GIANSANTI
Periodontitis (probably
related to defective leukocyte function)
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13. DIAGNOSIS
CLINICAL DIAGNOSIS
• Albinism
• Recurrent Infection
• Hepatosplenomegaly
• Oral Ulcerations and
• Periodontitis
LABORATORY DIAGNOSIS
• Hematological studies show presence of giant abnormal granules in the peripheral
circulating leukocytes, in their marrow precursors and in many other cells of the body.
• These granules are the hallmark of the syndrome.
• Thought to represent abnormal lysosomes
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14. MANAGEMENT
No specific treatment
Often fatal
Death occurring before child reaches 10
Antibiotics and drugs like Vincristine, Prednisolone and Ascorbic acid
have been tried for the treatment
14

15. INFECTIOUS
MONONUCLEOSIS(GLANDULAR FEVER)
• Also known as EBV infectious mononucleosis/ Pfeiffer's disease / Filatov's
disease
• Caused by Epstein Barr virus
• Drusenfieber (1889)
• First described by Sprunt and Evans in the John Hopkins Medical Bulletin(1920)
15

16. • Is transmitted by intimate contact with body secretions, primarily oro-
pharyngeal secretions and through deep kissing or intimate oral
exchange of saliva – so called as “kissing disease”.

17. CLINICAL FEATURES
Chiefly in Children and young adults
15-20 year age group
No sex or seasonal predilection
Most patients can be asymptomatic
Clinical syndrome consists of:
 Fever
 Pharyngitis
 Adenopathy
Tonsillitis, Headache, Chills, Cough, Nausea or Vomiting
Splenomegaly and Hepatitis
Enlargement of Cervical lymph node followed by the nodes of Axilla and Groin.
17

18. 18
Cervical lymphadenopathy Exudative pharyngitis

19. ORAL MANIFESTATIONS
FRASER AND MOODIE
• Acute gingivitis and stomatitis
• Appearance of white or gray membrane in various areas.
• Petechial hemorrhage of soft palate and occasional oral ulcers
• Edema of soft palate and uvula
SHIVER AND ET. AL. ( Emphasized on the petechial
COURANT AND SOBKOV hemorrhage of soft palate near the junction of hard palate
SCHUMACHER AND BARCAY an early diagnostic sign)
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20. 20
PETECHIAE ON THE PALATE WHITE OR GRAY MEMBRANE

21. Laboratory Findings
• Atypical lymphocytes in the circulating blood,antibodies to EBV and
increased heterophil antibody titre
• Positive Paul-Bunnell test(pathognomic and characteristic)
• Monospot test(highly specific test)

22. MANAGEMENT
No specific treatment
Bed rest and adequate diet
Short-term steroid therapy occasionally used
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23. LEUKEMIA
Disease characterized by the progressive over production of WBCs which
usually appear in the circulating blood in an immature form
Considered true malignant neoplasm(uncoordinated and independent
proliferation of WBC cells or their precursors)
Classified based on Clinical Behavior (Acute, Subacute or Chronic) and the
primary hematopoietic cell line affected (Myeloid or Lymphoid)
Acute: Survival less than 6 months
Chronic: Survival of over 1 year
Subacute: Survival duration lies between 6-12 months
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24. Classification of leukemias
Two major types (4 subtypes) of leukemias
Acute leukemias
Acute lymphoblastic leukemia (ALL)
Acute myelogenous leukemia (AML)
(also "myeloid" or "nonlymphocytic")
Chronic leukemias
Chronic lymphocytic leukemia (CLL)
Chronic myeloid leukemia (CML)
(Within these main categories, there are typically
several subcategories)

25. Acute vs. chronic leukemia
• Acute leukemias:
• Young, immature, blast cells in the bone marrow
(and often blood)
• More fulminant presentation
• More aggressive course
• Occurs more commonly in children and young adults
• Chronic leukemias:
• Accumulation of mature, differentiated cells
• Often subclinical or incidental presentation
• In general, more indolent (slow) course
• Frequently splenomegaly
• Mature appearing cells in the B. marrow and blood
• Occurs in adults of middle age or older

26. Phladiphia chromosome
Philadelphia chromosome is an acquired cytological
abnormality in the leukemia cells in CML
• Present in >80% of those with CML. It is a hybrid
chromosome comprising reciprocal translocation
between the long arm of chromosome 9 and the long
arm of chromosome 22—t(9;22) forming a fusion
gene BCR/ABLon chromosome 22, which has
tyrosine kinase activity

29. Distinguishing AML from ALL
• light microscopy
• AML: Auer rods, cytoplasmic granules
• ALL: no Auer rods or granules.
• flow cytometry
• special stains (cytochemistry) i.e
• AML:MPO +ve,SBB +VE,NSE +VE in M4,M5,M7,FINE PAS +VE IN
M6,M7
• ALL:BLOCK PAS +VE,ACID PHOSPHATASE +VE in T ALL

30. • Auer rods in AML • ALL

31. ETIOLOGY
Viruses:
Epstein Barr Virus, Herpes Virus and Human T-leukemia virus
Radiation and Atomic Energy:
When exposure is over the dose of 100 rads
Chemical agents:
Chronic exposure to aniline dyes, benzene and Phenylbutazone
Anti-cancer drugs like Melphalan and Cholorambucil have an increased risk of
developing leukemia especially Myelocytic variety.
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32. ACUTE LYMPOID LEUKEMIA
• Affects the children younger
than 15 years of age.
• Due to defect in lymphoid
cells differentiation.
• More common in whites
than non whites.
• M>F
• Do not have
myeloperoxidase positive
granules.
ACUTE MYELOID LEUKEMIA
• Affects between the
age of 15 to 39 years.
• Due to defect in
myeloid cells
differentiation.
• No such association.
• Have
myeloperoxidase
positive granules
along with auer rods.
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33. 33
ALL AML

34. CHRONIC MYELOID LEUKEMIA
• Malignancy involving
myelocytes
• Associated with
chromosomal
abnormalities ie.
Philadelphia
chromosome.
• Occurs between the age
of 30 to 60 years.
• Complete series of cells
including myeloblast,
promyelocyte,
metamyelocyte and
band cells are seen in
peripheral smear.
CHRONIC LYMPHOID LEUKEMIA
• Malignancy
involving
lymphocytes.
• No such association.
• M>F
• Occurs in the
patients above 45
years of age.
• Smudge cells are
the characteristic
feature.
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35. 35
CML CLL

36. CLINICAL FEATURES
Acute leukemia
• Abrupt stormy in onset
with pyrexia
• Weakness, fever,
headache, generalized
swelling of lymph node
• Petechial or ecchymotic
hemorrhages of skin
and mucous membrane
• Spleen, liver, kidney
enlarged
Chronic leukemia
• Insidious in onset
• Patient appears healthy
or may exhibit features
such as: anemic pallor
and emaciation
• Lymph node enlargement
is common in CLL than in
CML
• Enlargement of salivary
gland and tonsils
resulting in Xerostomia
• Petechiae / Ecchymoses
leading to nodular
lesions–skin
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37. LABORATORY FINDINGS
Acute leukemia
• Anemia and
thrombocytopenia
• Bleeding and
clotting time are
prolonged.
• The leukocyte
count rises in
terminal stage to
1,00,000/mm3.
Chronic leukemia
• Anemia and
thrombocytopenia.
• Leukocytosis upto
5,00,000/mm3 and
a very low white
blood cells are also
reported
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38. ORAL MANIFESTATIONS
 Oral lesions occur in both Acute and Chronic forms
 80% of affected patient exhibit Gingival Hyperplasia in Acute Monocytic Leukemia
 Gingivitis, hemorrhage, petechiae and ulceration of the mucosa
 Gingivae are boggy, edematous and deep red
 Gingivae bleed easily
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39. 39
GINGIVAL HYPERPLASIA
GINGIVAL HYPERTROPHY
GINGIVAL ENLARGEMENT

40.  Purpuric lesions of the oral mucosa seen
 Rapid loosening of the teeth seen (due to necrosis of PDL)
 Destruction of alveolar bone
 Osseous changes in the jaw including alterations in developing tooth crypts, destruction of Lamina Dura,
displacement of teeth
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41. 41
Ulceration of palate Crusting of lip

45. TREATMENT
• According to type of leukemia
• Treatment consists of supportive care, Chemotherapy, Radiotherapy, Corticosteroid therapy or Bone
marrow transplantation
• Chemotherapy includes three phases:
• Induction
• Consolidation
• Maintenance
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46. Leukemoid Reaction
• A leukemoid reaction describes a high
WBC count with neutrophilia,usually in
response to infection.
• The WBC count may be as high as 50,000
/microL and can easily mimic CML or
AML.

47. Features Suggesting Leukemoid Reaction
• Toxic granulation.
• High Neutrophil Alkaline Phosthotase (NAP) score.
• Presence of an obvious cause for the
neutrophilia.

48. TUMORS OF LYMPHOID TISSUE
TWO TYPES:
HODGKIN’S LYMPHOMA
NON-HODGKIN’S LYMPHOMA
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49. HODGKIN’S LYMPHOMA
• Lymphoproliferative disorders arising from lymph nodes and
from lymph components of various organs
• First described by British pathologist, Thomas Hodgkin in 1932
• Characterized by painless enlargement of lymphoid tissue
throughout the body
• Exact cause unknown
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50. ORAL MANIFESTATIONS
• Incidence:
Primary jaw lesion are uncommon
• Secondary effect:
Seen in oral cavity in the form of
infection due to reduced host immune
response
• Appearance:
Appear in the oral cavity as an ulcer
or a swelling or as an intra – bony lesion
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51. DIAGNOSIS
• CLINICAL DIAGNOSIS
Rubbery consistency of the enlarged
lymph node
• LABORATORY DIAGNOSIS
Differentiated from Non-Hodgkin’s
Lymphoma by the presence of cell known as Reed-
Stenberg Cells
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52. NON-HODGKIN’S LYMPHOMA
• Also called ‘LYMPHOSARCOMA’
• Neoplastic proliferation of lymphoid cells, usually affecting B-
lymphocytes
• Involves not only the lymph nodes but also bone marrow, spleen and
other tissues
• Early involvement of bone marrow is typical of this lymphoma
52

53. ORAL MANIFESTATIONS
• Site:
• Occurrence in oral cavity is rare; Found in tonsils, palate, buccal mucosa and gingiva
• Appearance:
• Palatal lesion are slow growing, painless, bluish soft tissue mass; often confused with minor salivary gland
• Symptoms:
• Paresthesia of MENTAL NERVE
• Pain and neuralgia of 2nd and 3rd division of Vth cranial nerve
• Signs:
• Necrotic proliferation, ulceration and discolouration of Palate
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54. NON-HODGKIN’S LYMPHOMA OF GINGIVA
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55. Non Hodgkin’s lymphoma in hard palate

56. HL NHL
1. A relatively homogeneous
disease process
2. Neoplastic cells are only
< 1% of the total cell
population in the lesion -
rest are reactive normal
cells.
3. LOCALIZED to start with
– CONTIGUOUS spread
from one nodal region to
another.
1. Extremely heterogeneous
2. With very rare exception,
neoplastic cells are always
the predominant cell
population often up to
100%.
3. SYSTEMIC to start with –
NON-contiguous spread.

57. HL NHL
4. Rare extra-nodal
involvement.
5. Rare GI / Waldeyer’s
involvement.
6. BM involvement –
significant therapeutic
importance.
7. Associated with T-cell
mediated immune
deficiency – mycobacteria,
fungal, viral, protozoal
infections.
8. Always treated – localized
cases may receive only RT.
4. Relatively common.
5. Relatively common.
6. Not significant in many
cases.
7. Associated with humoral
immune deficiency –
bacterial infections.
8. Indolent cases may remain
untreated for years – when
treated, it is systemic CT –
RT only adjunctive.

58. THANK YOU