Hematological & systemic disorders

1. Good
Morning
Dr.Ram

2. Dr.Ram

3.  Study of blood and blood forming tissues is
called as hematology.
Components of Blood:-
 Plasma
 55%
 Blood Cells
 45%
 Three types
 Erythrocytes/RBCs
 Leukocytes/WBCs
 Thrombocytes/Platelets
Dr.Ram

4.  Composed of hemoglobin
 Erythropoiesis
 = RBC production
 Stimulated by hypoxia
 Controlled by erythropoietin
 Hormone synthesized in kidney
 Hemolysis
 = destruction of RBCs
 Releases bilirubin into blood stream
 Normal lifespan of RBC = 120 days
Dr.Ram

5. ANEMIA:-
 Anemia is defined as abnormal reduction in the number of
circulating RBC,the quantity of hemoglobin & the volume of
packed red cells in a given unit of blood.
THALASEMIA:-
 Thalasemia is genetically determined disorder of hemoglobin
synthesis with decreased production of either alpha or beta
chains of hemoglobin.
POLYCYTHEMIA:-
 Abnormal increase in the number of RBC in the peripheral
blood, with an increased hemoglobin content
Dr.Ram

6. PATHOPHYSIOLOGIC CLASSIFICATION
1)Anemias due to increased blood loss.
Acute post hemorrhagic anemia
Chronic blood loss
2)Due to impaired cell function
a) cytoplasmic maturation defects:
Iron deficiency anemia
Thalassaemic syndromes
b) Nuclear maturation defects:
Vit B12 deficiency-Pernicious anemia
Megaloblastic anemia
c) Defects in stem cell proliferation: Aplastic anemia
Pure red cell aplasia
Dr.Ram

7. d)Anemia of chronic disorders
e)Bone marrow infiltration
f)Congenital anemia
3)Hemolytic anemias:Extracorpuscular red cell abnormalities
Intracorpuscular red cell abnormalities
MORPHOLOGIC CLASSIFICATION
1)Microcytic hypochromic anemia
2)Normocytic normochromic anemia
3)Macrocytic normochromic anemia
Dr.Ram

8.  It is common chronic hematologic disorder.
 Associated with deficiency of vitamin B12,due to lack of
production of intrinsic factor.
C/F:-
 Seen in individuals above 30 yr.
 F>M
 Triad of symptoms:
Generalized weakness
Sore & painful neck
Numbness or tingling of extremities
 Pt with severe anemia yellowish tinge of skin & sclera is
seen.
 Degeneration of peripheral nerves is seen.
Dr.Ram

9. Oral manifestations
 Glossitis is most common & early
manifestation of perinicious
anemia.
 Tongue is beefy red in colour
either completely or patches
scattered over dorsum & lateral
borders.
 Gradual atrophy of papilla of the
tongue resulting in smooth or bald
tongue(HUNTER’s or
MOELLERS GLOSITIS).
Dr.Ram

10. BLOOD PICTURE
 RBC <1,000,000/cumm.
 RBC are macrocytic &some times
poikilocytosis can be seen.
 Serum bilirubin is increased.
 Gastric secretions are increased &
intrinsic factor is either decreased or
completely absent.
 Bone marrow aspiration shows pre
erythrocytes with large nucleus &
chromatin in the form of clumps
(checker board appearance) .
 Giant platelets are seen in the smears
TREAMENT:-
-- Administration of vit B12 & folic
acid
-- Delayed treament leads to
neurological complications
Dr.Ram

11. Common type of anemia in females.
Etiology:-
Ch blood loss
Inadequate dietary intake
Faulty iron absorption
o Plummer–Vinson syndrome presents as a triad of dysphagia ,
esophageal webs, and iron deficiency anemia.
o It most usually occurs in postmenopausal women.
C/F:-
o Can occur at any age.
o F>M
o Angular chelitis
o Dysphagia to solid food
o Lemon tint pallor of skin
o Koilonychia
o Splenomegaly
Dr.Ram

12. Oral manifestations:-
 Glossitis-smooth red painful tongue with atrophy of filliform
& fungiform papillae.
 Mucous membrane of oral cavity & esophagus are atrophic
& show loss of keratinization ,which can lead to
development of cancer.
Blood picture:-
 Hypochromic,microcytic anemia.
 RBC count 3-4,000,000/cumm.
 Low Hb.
Treatment:-
 Responds to iron therapy &high protein diet.
 Dysphagia due to severe webs can be managed with
mechanical dialation.
Dr.Ram

13. SICKLE CELL ANEMIA
 Sickle cell anemia is autosomal dominant chronic hemolytic
anemia.
 The name is given due to microscopic appearance of sickle or
crescent shaped RBC in the circulating blood.
 In this adult hemoglobulin( HbA) is altered by the substitution of
glutamine with valine at 6th position of beta globulin chain.
 This produces sickle hemoglobin (Hbs) which gives sickle shape
to RBC .
 Life cycle of sickle-shaped RBC is very short-10-20 days.
Dr.Ram

14. C/F:-
 Common in females
 Age-below 30yrs
 Impaired growth
 Increased susceptibility to infections due to impaired
function of spleen
 Weak
 Short of breath
 Pain in joints limbs & abdomen
 Nausea& vomiting
 Systloic murmur & cardiomegaly
Dr.Ram

15. ORAL MANIFESTATIONS
 Mild to severe osteoporosis of
jaw bones with large irregular
marrow spaces
 R/F shows hair-on-end pattern of
trabeculae-perpendicular
radiating outward from inner
cortical plate.
BLOOD PICTURE
 RBC<1,000,000 or less/cumm.
 Decreased levels of Hb.
 Blood smear shows sickle shaped RBC.
TREATMENT
 Prevention & treatment of infections
 Management of pain
 Management of vasoocclusive crises
Dr.Ram

16.  Aplastic anemia is a bone marrow failure syndrome
characterized by peripheral pancytopenia & general lack of
bone marrow activity.
Two types:-
Primary aplastic anemia
Secondary aplastic anemia
Primary aplastic anemia is of unknown etiology.
 Effects young adults, develops rapidly & terminates fatally.
FANCONI’S SYNDROME:-
 Familial or congenital aplastic anemia
 Bone abnormalities
 Microcephaly
 Generalized olive-brown pigmentation of the skin
Dr.Ram

17.  Secondary aplastic anemia:-
 Known etiology
 Occurs at any age
Etiology:-
 Drugs or chemicals
 X-rays, radium or radio active isotopes
 Infections
C/F:-
 Severe weakness even with slight physical exertion
 Pallor of the skin
 Numbness & tingling of extremities
 Patechiae of skin & mucous membrane
 Decreased resistance to infections
Dr.Ram

18. Oral manifestations:-
 Petechiae & hematomas of oral mucosa
 Spontaneous gingival bleeding
 Ulcerative lesions of oral mucosa & pharynx
Blood picture:-
 RBC<1,000,000 or less/cumm
 Reduced Hb
 Thrombocytopenia
 Pancytopenia –hypoplasia of all marrow elements
 Severe-hypocellular bone marrow with fatty replacement.
Treatment:-
 Management of infections
 Bone marrow transplantation
 Immunosuppressive therapy
Dr.Ram

19.  Thalasemia is genetically determined disorder of hemoglobin
synthesis with decreased production of either alpha or beta
chains of hemoglobin.
 HbA=Heme +Globin(2 alpha & 2 beta chains)
 Thalasemia -decreased synthesis of either alpha or beta
chains.
 Alpha thalasemia –Alpha chain is deficient
 Beta thalasemia- Beta chain is deficient ,excess alpha chains
unstable hemoglobin.
Dr.Ram

20. Thalasemia minor:-mild ,effects both alpha & beta chains
Thalasemia major:-
 Production of beta chains is decreased.
 decreased synthesis of hemoglobin(hypochromic)
 Due to excess alpha chains inclusion bodies are formed with in
erythocytes (Fessas bodies).
 Leads to destruction of primitive red cell & severe ineffective
hemopoisis.
 70-80% of normoblasts are destroyed.
Hemoglobin H disease-mild –deficiency of alpha chains.
Hemoglobin bart’ diseases- still born infants
Dr.Ram

21. C/F-
 Beta thalasemia appears in first few
years.
 Child has yellowish palor of the
skin.
 Fever ,chills, generalized weakness.
 Protrusion of abdomen.
 Rodent facies-prominent cheek
bone, protrusion of max
anteriors,depression of bridge of the
nose.
 Degree of cephalofacial deformity
= severity of disease.
 Course of diseases is aggrevated
with infections.
 Pts may die with in few months.
 Thalasemia minor is with out any
clinical manifestations.
Dr.Ram

22. ORAL MANIFESTATIONS:-
 Prominence of premaxilla-
malocclusion.
 Anemic pallor of oral mucosa.
BLOOD PICTURE:-
 Hypochromoc & microcytic
anemia.
 RBC show poikilocytosis &
anisocytosis
 Nucleated RBC.
 Target cells are seen-
condensation of colour material
in the centre of red cell.
 Safety –pin cells.
Dr.Ram

23.  WBC <10,000-25,000.
 Bone marrow shows hyperplasia
with large number of immature
RBC.
 Serum bilurubin is elevated.
R/F:-
 Skeletal changes are characteristic.
 Rib with in rib at middle &
posterior portions of the ribs.
 Skull shows thickening of medulla
with poorly defined outer & inner
cortical plates, elongation of
trabeculae between cortices-crew
cut or hair–on-end appearance.
 IOPA shows thinning of lamina
dura.
 Peculiar trabecular pattern shows
salt & pepper appearance
Dr.Ram

24. .
TRETMENT:-
No treatment.
 Blood transfusions give temporary remission.
 Bone marrow trasplantation
 Disease is fatal & death can be due to infections, cardiac
damage because of anoxia or liver failure.
Dr.Ram

25. DISEASES OF WBC
Dr.Ram

26. White blood cells are cells of the immune system involved in
defending the body against both infectious disease and
foreign materials.
Two types –
Granulocytes:Neutrophills
Basophills
Eosinophills
Agranulocytes:Lymphocytes
Monocytes
Dr.Ram

27. Diseases of WBC
 Leukemia
 Agranulocytosis
 Neutropenia
 Infectious mononucleosis
Dr.Ram

28.  Leukemia is characterized by progressive production of
WBC,which circulate in blood in immature form.
 It is a true malignant which is often fatal .
 Any of WBC can be involved.
CLASSIFICATION
Based on course of disease:
Acute leukemia
sub acute leukemia
Chronic leukemia
Dr.Ram

29. •Based on cell it involves:
• Lymphoid leukemia-
•Involves lymphocytic series
• Myeloid leukemia-
•Involves progenitor cells that
terminally differentiate into cells of
myeloid series
Dr.Ram

30. ETIOLOGY:-
 Etiology of leukemia is unknown.
 Can be infectious origin-Ebstein-Bar virus
Human T-cell leukamia virus-1
 Ionizing radiation
 Ch exposure to chemicals like benzol,aniline dyes.
Chromosomal abnormalities-
 philadelphia chromosome
 Seen in 85-95% of ch myeloid leukemia
 Translocation of chromosomal material from chromosome 22
to 9.
 Downs syndrome is due to trisomy of chromosome 21
Dr.Ram

31. C/F:-
 Acute leukemia-children & young adults
 Ch leukemia-middle age or older age
 M>F
Acute leukemia:-Acute lymphoblastic leukemia(ALL)
Acute myeloblastic leukemia(AML)
 Leukemic cells develop from immature blast cells
 Weakness,fever, head ache.
 Swelling of lymph nodes-first sign.
 Petechial & echymotic hemorrhages in skin & oral mucosa.
 Splenomegaly& hepatomegaly due to leukemic infiltration.
 Hemorrhages due to decrease in platelets.
 Infections due to crowding myeloid tissue.
Dr.Ram

32. Chronic leukemia:-2 types:CML-20%
CLL
 Cells involved are mature cell.
 Slow before symptomatic.
 Found on routine hematological examination.
 Anemic pallor & emaciation.
 Lymphadenopathy-ch lymphoblastic leukemia.
 Sphenomegaly & hepatomegaly.
 Enlargement of salivary glands & tonsils-xerostomia
 Skin lesions-papules, pustule, bullae, areas of pigmentation,
nodular lesions having leukemic cells.
 Destructive lesions of bone –ch leukemia-pathologic fracture
& osteomyelitis.
Dr.Ram

33. ORAL MANIFESTATIONS
 Common in acute monocytic leukemia.
 Absent in young & edentulous individuals.
 Least in acute lymphocytic leukemia.
 Gingivitis.
 Gingival hyperplasia-gingiva is edematous, boggy,& deep
red.
Gingiva bleeds easily
In severe teeth are completely hidden.
 Gingival hemorrhage due to ulceration& necrosis of
underlying tissue.
 Moblity of teeth due to necrosis of PDL & destruction of Al
bone.
Dr.Ram

34. Blood picture
Acute leukemia:-
o Develop from blast cells.
o Anemia & thrombocytopenia
o Prolonged bleeding & clotting
time
o Platelet count is less than
50,000/cumm.
o WBC-100,000/cumm.
o In myeloid leukemia-
undifferentiared myelocytes
predominates
Stem cell leukemia-
cells are highly undifferentiated
Dr.Ram

35. Chronic leukemia:-
o Leukemic cells develop from
more mature cells.
o CML-Basophils-10%
o Anemia & thrombocytopenia
o WBC-5,00,000/cumm
o Some times low WBC counts.
o Has better prognosis than acute
leukemias.
Dr.Ram

36. TREATMENT
 Chemotherapy
 Radiotherapy
 corticosteroids
Dr.Ram

37.  Also known as glandular fever or kissings disease.
 It is an infectious, widespread viral disease caused by the
Epstein–Barr virus(EBV).
 Incubation period-4-7weeks.
 It is a clinical syndrome characterized by fever,pharyngitis
and adenopathy.
C/F:-
 Age-15-20yrs.
 Fever ,chills ,head ache.
 Sore throat, cough, nausea & vomoting.
 Bilateral & symmetrical lymphadenopathy.
 Sphenomegaly & hepatits.
 Pharyngitis & laryngitis.
Dr.Ram

38. ORAL MANIFESTATIONS
 Acute gingivitis & stomatitis.
 Palatal patechiae at the junction of hard & soft palate is the
early manifestation.
 Lesions persist for 3-11 days & fade gradually.
 Edema of soft palate & uvula.
 Gingival & oropharyngeal bleeding is seen.
Dr.Ram

39. LABORATORY FINDINGS:-
 Atypical lymphocytes , resembling monocytes.
 Antibodies to EB virus.
Positive paul- Bunnel test-
 A test for the presence of heterophile antibodies in the serum produced
in infectious mononucleosis; agglutination of sheep red cells is a
positive test.
 ESR is high.
 Thrombocytopenia.
TREATMENT:-
 Bed rest & adequate diet.
 Steroid therapy can be given.
 Disease persist for 2-4 weeks .
Dr.Ram

40.  Agranulocytosis is the disease involving WBC which is
characterized by decreased number of circulating
granulocytes.
Two types ;-
 Primary agranulocytosis- un known etiology.
 Secondary granulocytosis
Etiology:-
 Infections
 Hemopoitic disorders
 Chemical agents
 Physical agents
 It mainly in individuals who manifest allergic reactions .
Dr.Ram

41. Mechanism:
 In drug induced agranulocytosis the drug may act as antigen &
induce antibody formation.
 These antibodies destroy granulocytes or form immunocomplexes
which bind to neutrophils & destroy them.
 KOSTMANN SYNDROME: Inherited autosomal recessive
pattern in which severe congenital neutropenia is seen.
C/F:-
 Can occur at any age.
 F>M
 Mainly affects health workers .
Dr.Ram

42.  Disease commences with high fever, chills & soar throat.
 Skin pale & anemic some times yellow tinged.
 Characteristic feature is presence of infections mainly in oral
cavity , GIT, GUT,RT,& skin.
 Regional lymphadenitis .
 If not treated in time infection can become generalized
sepsis& may be fatal.
 Clinical symptoms develop rapidly with a few days & death
may occur with in a week.
Dr.Ram

43. O/M:-
 Necrotizing ulcerations of oral mucosa mainly palate &
gingiva, tonsils ,pharynx.
 Lesions appear as ragged necrotic ulcers covered by gray or
black membrane.
 Necrosis of gingiva begins adjacent to sulcus -free gingiva -
PDL -Al bone.
 Excessive salivation
 Oral surgical procedures are contraindicated.
Dr.Ram

44. Laboratory findings:-
 WBC below 2000/cumm.
 Complete absence of granulocytes.
 Bone marrow shows myeloblasts, premyelocyte,
metamyelocytes & myelocyte.
Treatment:-
 Recognition & with drawel of causative agent.
 Administration of antibiotics for the control of infections.
 Agranulocytosis secondary to viral diseases is self limiting &
has good prognosis.
Dr.Ram

45.  Cyclic neutropenia is an unusual form of agranulocytosis
characterized by periodic diminution in circulating PNL.
 Rare congenital neutropenia.
 Etiology is unknown.
 Can be an autosomal dominant condition due to germline
mutations in GRANULOCYTE –COLONY STIMULATING
FACTOR. (G-CSF).
C/F:-
 Can occur at any age ,infants & young adults.
 Fever, sore throat, stomatitis,& regional lymphadenopathy.
 Head ache, arthritis,cutaneous infection, conjunctivitis can be
seen.
 The cycle will be ussually for 3 weeks, so there won’t be
significant bacterial infection.
Dr.Ram

46. O/M:-
 Patients exhibit severe gingivitis,somatitis with ulceration,
during the period of neutropenia.
 With neutrophil count normal the oral mucosa assumes
normal clinical appearance.
 In children repeated infection leads to loss of supporting
bone of the teeth.
 Isolated painful ulcers occur which persist for 10-14 days &
heal with scarring.
Dr.Ram

47. R/F:-
 IOPA shows severe loss of superficial alveolar bone, due to repeated cyclic
gingivitis ,advancing to periodontitis.
 Loss of bone around teeth in children is termed as prepubertal
periodontitis.
LABORATORY FINDINGS:-
 Pt exhibits normal blood count for a period of 4-5 days followed by
decrease in granulocytes for a period of 3weeks or for several months.
 At height of disease neutrophils may completely disappear for 1 or 2days.
Dr.Ram

48. Treatment & prognosis:-
 No specific treatment is necessary,
 Prognosis is better than typical agranulocytosis.
Dr.Ram

49. Dr.Ram

50. PLATELETS
o Platelets, or thrombocytes are small, irregularly shaped
cells, 2–3 µm in diameter.
o Platelets are derived from megakaryocytes.
o Normal count is 1,50000-4,00000
o The average lifespan of a platelet is 5 to 9 days.
o Platelets provide lipoprotein surface for the conversion of
prothrombin to thrombin.
Dr.Ram

51.  Thrombin helps in conversion of fibrinogen to fibrin which
causes the aggregation of platelets.
 Platelet disorders are the most common cause of
bleeding.
 The disorder could be decrease in the number
(thrombocytopenia) or defective function.
Dr.Ram

52. Diseases of platelets are:-
 Thrombocytopenic purpura
 Thrombocytopathic purpura
 Thrombocytosis
Dr.Ram

53.  Thrombocytopenia is a disease which is characterized by
abnormal reduction in number of circulating platelets.
 Purpura is defined as purplish discolouration of skin or
mucous membrane due to spontaneous extravassation of
blood.
 Purpura can be due to reduction in platelets or increased
capillary fragility.
Dr.Ram

54.  Based on the etiology purpura is of 2 types:-
- Non thrombocytopenic purpura –due to increased
capillary fragility.
- Thrombocytopenic purpura
Thrombocytopenic purpura is of 2 types:-
Primary thrombocytopenic purpura –unknown etiology
Secondary thrombocytopenic purpura
Dr.Ram

55. Etiology:-
.1. Failure of platelets production most common cause,
Megakaryocytes are  in the bone marrow
e.g. Ionizing radiation
Drugs & chemicals
Aplastic anemia
Infections
2.  rate of removal of platelets from the circulation
Drugs
Infections
Hemolytic anemia
3.Diceases causing excessive utilization of platelets
Splenomegaly
Platelet sequestration
Intravascular coagulation
Dr.Ram

56. Primary thrombocytopenic purpura :
 It is thought to be an autoimmune disorder in which a person
develops anitibodies to his own platelets.
 It can also be due to absence of platelet stimulating or
megakaryocyte –ripening factor.
 Acute form occurs in children following some viral
infections.
 Chronic form occurs mostly in adult females.
Dr.Ram

57. C/F:
 Secondary TP can occur at any age.
 Spontaneous appearance of purpuric or
hemorrhagic lesions of the skin ranging
from pinpoint petechiae to large
purpulish echymosis.
 Pt exhibits bruising tendency.
 Epitaxis
 Bleeding from urinary tract-hematuria.
 Bleeding in GIT –malena hematemesis.
 Intracranial hemorrhage - hemiplegia.
Dr.Ram

58. O/M:-
 Severe & profuse gingival
bleeding.
 Petechiea occur most
commonly on the palate.
 Echymosis occurs rarely.
Dr.Ram

59. Laboratory findings
 Platelets >60,000/cumm
 BT-more than one hr.
 CT is normal but clot shows failure of retraction.
 Capillary fragility is increased
 Giant platelets in peripheral smears-congenital
thrombocytopenia
Dr.Ram

60. Treatment & prognosis:-
 No specific treatment
 Splenectomy can be done.
 Corticosteroids
 Pt shows fairly good prognosis with intervals of remission &
exacerbation
 Death may be due sudden ,severe hemorrhage.
 Oral surgical procedures are contra indicated until deficiency
is cleared
Dr.Ram

61. Dr.Ram

62.  Hemophilia is characterized by prolonged coagulation time
& hemorrhagic tendencies.
 It is a heriditary disease carried by X- chromosome .
 It is transmitted as a gender- linked mandolin recessive trait.
 Females act as carriers ,rarely affected by diseases.
Dr.Ram

63. ETIOLOGY:-
Mainly 3 types of hemophilia.
 Hemophilia A-factor VIII or Anti hemophilic
globulin deficiency(AHG).
 Hemophilia B-IX plasma thromboplastin
component (PTC).christmas disease.
 Hemophilia C-XI plasma thromboplastin antecedent
(PTA)
Dr.Ram

64.  Genes for AHG & PTC are located on long arm of X
chromosome in q28 & q27.
 Mutations in these genes can lead to hemophilia.
 Gene for PTA is located on chromosome 4.
 Hemophilia A is the most common type & is also called as
classic hemophilia.
Dr.Ram

65.  AHG is a glycoprotein which has 3 components.
1)clot promoting factor-
corrects the coagulation defects in pt with classic
hemophilia.
2) factor VIII antigen-
present in pt with classic hemophilia & absent in pt
with Von Willebrand’s disease.
3) Von Willebrand factor-
synthesized by endothelial cells which corrects platelet
adhesion defects in Von Willebrand’s disease.
Dr.Ram

66. Based on the severity of disease divided into 3 types-
 Mild hemophilia-hemorrhage is due to major trauma
or surgery.
 Moderate hemophilia-mild to moderate trauma.
 Severe hemophilia-spontaneous & soft tissue
bleeding ,hemarthrosis.
Dr.Ram

67. C/F-
 Usually present by birth.
 50% of pts present with neonatal
bleeding such as bleeding from
umbilical cord.
 Persistent bleeding either
spontaneously or on minor trauma.
 Hemorrhage occurs into
subcutaneous tissues & internal
organs which leads to the formation
of massive hematomas.
 Bleeding into joints & muscles is
not present in Hemophilia C.
Dr.Ram

68. O/M:-
 Gingival bleeding is massive & severe.
 Even physiologic process of tooth erruption &
exfoliation can lead to severe & prolonged bleeding.
 Due to subperiosteal bleeding & reactive new bone
formation there will be formation of tumour like
expansion of mandible- pseudotumour.
Dr.Ram

69.  Surgical procedures should be carried out with proper
premedication.
 Tooth extractions can be carried out by means of rubber bands.
 The rubber band is placed around cervical portion of the tooth
& allowed to migrate apically, causing exfoliation of tooth
through pressure necrosis of periodontal ligament.
Dr.Ram

70. LABORATORY FINDINGS:-
 C .T –increased
 B.T ,prothrombin time –normal
 Plasma of hemophilic pts show deficiency of clot –
promoting factor.
Treatment :-
 Pts should be protected from traumatic injuries.
 Preoperative transfusion of whole blood & administration of
antihemophilic factor concentrate is recommended.
 Prognosis is variable ,& pts may die in childhood.
Dr.Ram

71.  Also known as pseudohemophilia or vascular hemophilia.
 It is characterized by excessive bleeding in pts with normal
platelet count, normal CT,normal serum fibrinogen & normal
prothrombin time.
 BT is prolonged.
 It is hereditary disease inherited as an autosomal dominant trait.
 Transmitted & manifested by both males & females ,but females
are most commonly effected.
 Caused by abnormalities of Von Willebrand factor(VWF).
Dr.Ram

72.  vWF is a large glycoprotein that acts as a carrier protein for
factor VIII.
 It helps in platelet aggregation (primary hemostasis) &
prevents the degradation of factor VIII & delivers it to the
site of injury.(secondary hemostasis).
 vWF gene is present on chromosome 12.
Dr.Ram

73. vWD is classified into 3 types:-
Type 1-
 partial decrease in vWF & factor VIII.
Type 2-
 qualitative defects in vWF.
Type 3-
 Severe & rarest form.
 Deficiency 0f both vWF & factor VIII in the plasma .
 vWF is absent in both platelets & endothelial cells.
 Severe clinical bleeding, inherited as an autosomal recessive trait
Dr.Ram

74. C/F:-
 Acquired form is seen in willim’s tumour & systamic lupus
erythmatosis.
 Most of the children are asymptomatic .
 Diagnosed on routine hematological examination.
 Excessive bleeding either spontaneously or on minor trauma.
 Bleeding from nose ,gingiva & skin.
 Bleeding into GIT common.
 Hemarthrosis is rare .
 Bleeding tendency may be spontaneous or cyclic.
Dr.Ram

75. O/M:-
 Bleeding of gingiva may be spontaneous or followed by minor
trauma.
 Disease may be discovered after extraction due to prolonged &
excessive bleeding following procedure.
Laboratory findings:-
 BT is prolonged- min - hrs.
 Prothrombin time & CT are normal.
 Capillary fragility is increased & positive tourniquet test.
 Poor platelet adherence.
Dr.Ram

76. Treatment:-
 Transfusion of plasma/ AHF.
 Pts may become refractory after repeated
tranfussions ,can develop antibodies to AHF.
Dr.Ram

77. Dr.Ram