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Neville and Damm > CH13- Hematologic Disorders (non-neoplastic) > Flashcards

CH13- Hematologic Disorders (non-neoplastic) Flashcards

1
Q

Most common head and neck site for lymphoid hyperplasia

A

anterior cervical chain

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2
Q

Most common locations for lymphoid hyperplasia in the oral cavity

A

oropharynx, soft palate, lateral tongue and floor of mouth

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3
Q

A deep nodule of intraoral lymphoid hyperplasia appears pink, versus a superficial lesion may appear ___________

A

yellow-orange

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4
Q

Enlarged, rubbery-firm, nontender, freely moveable lymph node most likely represents

A

chronic inflammation –> lymphoid hyperplasia

R/o lymphoma by clinical hx (not rapidly enlarging)

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5
Q

What condition can cause multiple chronically enlarged non-tender lymph nodes

A

HIV

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6
Q

Histology of lymphoid hyperplasia

Differential based on histo?

A

sheets of well-diff lymphocytes with germinal center formation

*differential may include follicular lymphoma

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7
Q

Hallmark feature of hemophilia (of all kinds)

A

increased coagulation time

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8
Q

3 most commonly encountered bleeding disorders

A

Hemophilia A, Hemophilia B, von Willebrand disease

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9
Q

Most common inherited bleeding disorder

A

Von willebrand disease

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10
Q

Another name for hemophilia A

A

classic Hemophilia

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11
Q

Prevalence of:
Hemophilia A
Hemophilia B
Von willebrand disease

A

A: 1:10,000 person, 1:5000 males
B: 1:60,000 persons, 1:30,000 males
VWD: 1:800-1000 people

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12
Q

% of cases of Hemophilia A that represent new mutations

A

30%

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13
Q

Mutations in what causes hemophilia A?

A

Factor 8- over 900 different mutations

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14
Q

Patients with ?% normal F8 can live relatively normally while those with ?% bruise with even minor trauma

A

25%
<5%
(Hemophilia A)

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15
Q

Definition: deep hemorrhage resulting in a tumor like mass (can occur intraorally)

A

Pseudotumor of hemophilia

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16
Q

Deep hemorrhage of muscle, soft tissue and the joints may occur in

A

Hemophilia

joints= hemarthrosis

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17
Q

What disease accounts for 80-85% of bleeding disorders related to a specific clotting factor

A

Hemophilia A

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18
Q

Inheritance pattern for:
Hemophilia A
Hemophilia B
Von willebrand disease

A

X linked recessive
X linked recessive
Autosomal: rec or dom

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19
Q

What lab value is abnormal in Hemophilia A & B

A

PTT

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20
Q

Abnormal lab values for VWD

A

Abnormal PFA(platelet function assay), abnormal PTT

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21
Q

What is the function of von willebrand factor

A

glycoprotein in the plasma that helps platelets adhere to a bleeding site, also a transport molecule for Factor 8 in the plasma

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22
Q

Hemophilia A:
>____% of factor 8 = probable normal function
_____% Factor 8 may be mild disease

A

> 25%
5-40%= no treatment for normal activity, maybe treatment for surgery
<1%-need injections of clotting factors as soon as hemorrhagic event occurs to prevent crippling deformities

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23
Q

AVOID ___ containing medications in hemophilia and VWD

A

aspirin

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24
Q

Antifibrinolytic agent given before and after surgery in hemophilia and VWD

A

EACA- epsilon-aminocaproic acid

give 1 day before and 7-10 days after

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25
Q

What drug causes release of bound factor 8. This is given to hemophilia patients with ____% F8

A

desmopressin (DDAVP)

> 5% factor 8

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26
Q

DDAVP is used in the treatment of

A 
Hemophilia A (>5% F8 available)
VWD type 1(most common type 70-80%)
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27
Q

Pre purification ___% hemophiliacs infected with HIV, ___% hepatitis C

A

80-90

40

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28
Q

What percent of hemophilia A patients develop autoantibodies to Factor 8

A

25-30%

occurs less freq with Factor 9

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29
Q

Hemophilia B is also called ______, it a deficiency in ___

A

Christmas disease

factor 9

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30
Q

2 other names for plasminogen deficiency

A

ligneous conjunctivitis and hypoplasminogenemia

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31
Q

Inheritance pattern for ligneous conjunctivitis / hypoplasminogenemia

A

autosomal recessive

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32
Q

What disease is characterized by irregular fibrin deposited in plaques and nodules primarily in the mucosal surfaces

A

plasminogen deficiency

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33
Q

involvement of the eyes by plasminogen deficiency is termed

A

ligneous conjunctivitis= “woodlike”

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34
Q

Clinical appearance of plasminogen deficiency lesions

A

Thick creamy yellow-erythematous firm plaques and nodules

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35
Q

T/F plasminogen deficiency more commonly affects men

A

FALSE- women

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36
Q

Hypoplasminogenemia: Ocular lesions in ____%, oral in ___%, respiratory tract in ___%, vaginal lesions in ___%

A

80%
34%
16%
8%

(about half each time)

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37
Q

Most common site for a lesion of plasminogen deficiency in the oral cavity

A

gingiva-patchy ulcerated papules with irregular surface that wax and wane

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38
Q

The amyloid-like material seen on histology of plasminogen deficiency is negative for amyloid stains and positive for _________?

A

fraser-lendrum IHC

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39
Q

T/F patients with plasminogen deficiency are more prone to thrombus

A

surprisingly these patients don’t have thrombus formation problems, life expectancy is normal

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40
Q

treatment of oral lesions of plasminogen deficiency

A
  • Topical heparin and prednisone combined

- Surgical excision + systemic doxy, topical chlorhexidine, systemic warfarin

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41
Q 
Major divisions of Anemia
1. Disturbed iron metabolism
2. \_\_\_\_\_\_ anemia
3. Anemia of \_\_\_\_\_\_\_
4. \_\_\_\_\_Anemia
5.
A
  1. Megaloblastic: Vitamin B12 def, folic acid def
  2. anemia of chronic disorders: infection, inflammatory CT dz, 2/2 malignancy, liver dz etc
  3. Hemolytic anemia: extrinsic and intrinsic
  4. Hemoglobin disorder aka Hemoglobinopathies: sickle cell, thalassemia
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42
Q

What amino acid change occurs to form Hemoglobin S

A

glutamic acid –> valine

Sickle cell disease

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43
Q

Patients with _____ have only ____% of hemoglobin affected and have sickle cell trait

A

1 mutated copy

40-50%

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44
Q

What % of the black population has sickle cell trait

A

8%

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45
Q

prevalence of sickle cell disease in blacks

A

1:350-400

46
Q

how long are sickle cell erythrocytes in circulation

A

12-16 days

47
Q

What are 3 most commonly affected sites in sickle cell anemia? What is acute chest syndrome?

A

Ischemic pain in 1. abdomen, 2. long bones and 3. lungs that occurs in sickle cell crisis…acute chest syndrome = pain in the lungs usually precipitated by a fat embolism or community aquired pneumonia

48
Q

What is a common cause of death among children with sickle cell disease and thalassemia? What are the prophylaxis recommendations?

A
  • strep pneumoniae infections
  • they are more susceptible likely due to multiple splenic infarcts-
  • Sickle cell: prophylactic penicillin antibiotics until age 5 and polyvalent pneumococcal vaccinations recommended
49
Q

What % of children have a stroke by 8 years old as a result of sickle cell disease

A

5-8%

50
Q

Oral findings in sickle cell disease (radiographic)

A

reduced trabeculation of the bone (2/2 increased hematopoiesis), increased risk osteomyelitis, infarction, asymptomatic pulpal necrosis

51
Q

“HAIR ON END” radiographic appearance is described in what condition (s)? Which condition is it more prominantly seen in?

A

sickle cell anemia

thalassemia (more prominant)

52
Q

sickle shaped RBCs are also called ____ shaped

A

boomerang

53
Q

What is the affect of hydroxyurea? What is a curative, albeit not usually feasible treatment for sickel cell?

A

increases fetal hemoglobin (Hemoglobin F) in adult patients with sickle cell (inhibits polymerization of hemoglobin S and reduces adherence of RBCs to vessel walls)….CURATIVE = bone marrow transplant, but need HLA match with donor sibling - only 1% qualify

54
Q

carriers of sickle cell trait and thalassemia are more resistant to _____… what is the root of the word ‘thalassemia’?

A

malaria…thalass = sea in Greek (Mediterranean Sea is where the first reports originated)

55
Q

1 of the most common inherited conditions that affect humans…what are the two types of anemia that arise of this?

A

Thalassemia = 1. hypochromic 2. microcytic anemia

56
Q

___ genes code for the beta chain; ____ genes code for the alpha chain of hemoglobin…how many mutations have been documented for b-thalassemia?

A

2 beta, 4 alpha…more than 200 different mutations

57
Q

1 beta chain mutated –>

2 beta chains mutated —> ? What are the 2 alternate names?

A

thalassemia minor

thalassemia major aka 1. Cooley’s anemia or 2. Mediterranean anemia

58
Q

Description of the type of anemia in beta thalassemia major after the patients fetal hemoglobin dissipates

A

Severe microcytic hypochromic anemia (occurs at 3-4 months)

59
Q

Hematopoiesis to compensate in beta thalassemia major increases by _____ = massive marrow hyperplasia, hepatosplenomegaly, LAD

A

30x

60
Q

“CHIPMUNK” face occurs in what condition and is a manifestation of what

A

occurs in beta thalassemia major

-painless maxilla and mandibular enlargement caused by massive marrow hyperplasia

61
Q

1 deleted gene →
2 deleted genes →
3 deleted genes →
4 genes deleted (homozygous) →

A

1–>no detectable disease
2–>alpha thalassemia trait
Mild anemia and microcytosis. Usually not clinically significant
3–> hemoglobin H (HbH) disease. Hemolytic anemia and splenomegaly
4–>hydrops fetalis = severe generalized fetal edema

62
Q

How often do thalassemia major patients need transfusions? Significant side effect from blood transfusions

A

every 2-3 weeks…hemochromatosis: iron overload

63
Q

What medication is given to help combat hemochromatosis in thalassemia patients receiving transfusions?

A

deferoxamine (iron chelating agent)

64
Q

Pancytopenia occurs in what condition?

A

aplastic anemia

failure of hematopoietic precursor cells to make all types of blood cells (cytotoxic t-cell mediated reaction)

65
Q

Etiology of aplastic anemia

A

t cell are targeting the precursor cells in the bone marrow

66
Q

Causative/inciting agents of aplastic anemia

A

toxins- benzene
drugs- chloramphenicol
infections- ABCG types of hepatitis

67
Q

2 Genetic disorders with an increased risk of aplastic anemia

A

Fanconi Anemia

Dyskeratosis congenita

68
Q

6 Oral changes seen in aplastic anemia

A

Petechiae, purpura, gingival hemorrhage
Pale oral mucosa from decreased RBCs
Oral ulcers from infections/neutropenia
Gingival hyperplasia

69
Q

In a biopsy of an oral ulcer in aplastic anemia what would you see

A

organisms and a LACK of inflammatory response

70
Q

Diagnostic criteria for aplastic anemia

A

based on labs: 2 or more:

  • Fewer than 500 granulocytes/uL
  • Fewer than 20,000 platelets/uL
  • Fewer than 20,000 reticulocytes/uL
71
Q

patients with aplastic anemia are at an increased risk for what malignancy

A

acute leukemia

72
Q

Neutropenia is defined as circulating neutrophils below ______

A

1500/mm3

73
Q

patients of African and mediterranean backgrounds can have normal neutrophil levels around 1200, this is called

A

benign ethnic neutropenia

74
Q 
Causes of neutropenia in infants are predominantly congenital or genetic such as:
1.
2.
3.
4.
A
  • Schwachman-Diamond syndrome
  • Dyskeratosis congenita
  • Cartilage-hair syndrome
  • Severe congenital neutropenia
75
Q

6 Causes of neutropenia in adults…What is the most common type of infection to cause neutropenia?

A
  • destruction of BM by malignancy
  • destruction from metabolic diseases: Gaucher disease
  • osteopetrosis
  • drugs: chemo, antibiotics, phenothiazines, tranqs, diuretics
  • infections: Staph aureus (or bacterial) infection = most common
  • SLE
76
Q

Gaucher disease

A

auto recessive disorder where fat-laden gaucher cells build up in spleen, liver, and bone marrow

77
Q

What is a Gaucher cell

A

histiocytes filled with linear or fibrillar material called kerasin

78
Q

Neutropenia in SLE is caused by

A

anti-neutrophil antibodies

79
Q

Viral and bacterial infections _____ neutrophil production and ______ ______ ex. HIV, hep A&B, measles, rubella, varicella, TB, typhoid etc

A

reduce production anddd increase destruction

80
Q

Patients with neutropenia are prone to bacterial infections, particularly ________ infection

A

staph aureus

81
Q

Most common sites of infection in neutropenia

A

peri-rectal, oral cavity, middle ear

–>lung when it drops below 500 count

82
Q

Oral cavity manifestation neutropenia: ulcers of the ______ that characteristically ______

A

gingiva; lack an erythematous border

83
Q

A major treatment for neutropenia

A

G-CSF
granulocyte colony stimulating factor
(filgrastim, pegfilgrastim)

84
Q

Agranulocytosis affects all the cell of the granulocyte lineage, but particularly _______

A

neutrophils

85
Q

Etiology agranulocytosis

A

mostly drug induced

sometimes congenital

86
Q

2 congenital causes of agranulocytosis (decreased level of G-CSF)

A
  • Congenital agranulocytosis

- Kostmann syndrome (severe congenital neutropenia)

87
Q

In aplastic anemia and agranulocytosis the loss of ______ is more impactful than other cell absences

A

neutrophils

88
Q

The oral manifestation in agranulocytosis may mimic

A

NUG on the gingiva

89
Q

Necrotizing deep punched out ulcerations

A

oral presentation in agranulocytosis

90
Q

If drug induced _______ usually resolves within 10-14 days after the offending drug is removed

A

agranulocytosis

91
Q

Disease course of cyclic neutropenia

A
  • Idiopathic regular periodic reductions in the neutrophil count of affected patients
  • drop lasts 3-6 days
  • Uniformly spaced episodes: every ~21 days
92
Q

What mutation is implicated in cyclic neutropenia? Which stage is development of the neutrophil arrested?

A

neutrophil elastase gene

ELA-2 aka ELANE….PROmyelocyte stage

93
Q

prevalence of cyclic neutropenia? Inheritance? What is the typical length of the neutrophil cycle?

A

1:1 million..AD (but most cases are isolated)…every 21 days

94
Q

What disease? Oral ulcers occur anywhere with minor trauma, erythematous halo is variably present. Severe periodontal bone loss and gingival recession

A

cyclic neutropenia

95
Q

Neutrophil count falls to ____ for 3-5 days in at least 3 successive cycles for diagnosis. DX?

A

500

cyclic neutropenia

96
Q

treatment cyclic neutropenia

A

G-CSF several times a week. Reduces the drop in neutrophils from 5-6 days to 1 day.

97
Q

When do the symptoms of cyclic neutropenia naturally diminish

A

second decade of life, despite the continued cycling

98
Q

What causes Thrombotic Thrombocytopenic purpura (TTP)

A

Deficiency in von willebrand factor cleaving metalloprotease (ADAMTS13) (or AR recessive ADAMTS13 gene mutation)

TTP: Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in which many small blood clots (thrombi) form suddenly throughout the body. it uses up all your platelets and that is why they are low.

99
Q

Deficiency in von willebrand factor cleaving metalloprotease aka ADAMTS13 is caused by

A

autoantibodies to ADAM or inherited mutations to ADAM

ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13)—also known as von Willebrand factor-cleaving protease (VWFCP)—is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor

100
Q

Basic mechanisms of thrombocytopenia

A
  • Decreased production
  • Increased destructions
  • Increased use of platelets in abnormal clotting disorders
  • Platelet trapping in the spleen in splenomegaly
101
Q

thrombocytopenia is usually not clinically detected until platelets are below ______

A

100,000

normal 200,000-400,000

102
Q

Acute ITP: Immune(previously idiopathic) thrombocytopenic purpura occurs in what age group

A

Childhood

103
Q

Acute ITP usually resolves spontaneously in _____ weeks. ___% of pts recover in 3-6 months

A

4-6 weeks

90%

104
Q

Chronic ITP: Immune(previously idiopathic) thrombocytopenic purpura occurs in what age group

A

20-40 year old women

105
Q

Cause of chronic ITP

A

Autoantibodies to platelet surface antigens leads to sequestration of the platelets in the spleen
Treated with splenectomy

106
Q

What condition? 30-40% of oral biopsy show fibrin deposits in small vessels (PAS stain)

A

thrombocytopenia

107
Q

3 Other names for: Polycythemia vera

A

(primary polycythemia, polycythemia rubra vera; primary acquired erythrocytosis)

108
Q

Polycythemia vera is defined as

A

an increase in the MASS of the RBCs (also freq uncontrolled production of platelets and granulocytes too)

109
Q

What is the aquired mutation in polycythemia vera?

A

JAK2 (janus kinase 2)

110
Q

What is the peripheral vascular event assoc with polycythemia vera?

A

erythromelalgia

111
Q

What % of polycythemia vera patients develop leukemia?

A

2-10%

Neville and Damm (72 decks)

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