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OP - Yield > 13: Hematologic disorders > Flashcards

13: Hematologic disorders Flashcards

1
Q

hemophilia A factor

A

factor VIII deficiency

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2
Q

factor VIII deficiency

A

hemophilia A

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3
Q

hemophilia B factor and aka

A

factor IX deficiency

aka christmas dz

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4
Q

factor IX deficiency

A

hemophilia B

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5
Q

deep hemorrhage in muscles, soft tissues, joints

A

hemarthrosis

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6
Q

hemorrhage in hemophilia causing a tumor like mass

A

pseudotumor of hemophilia

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7
Q

plasminogen deficiency inheritance and mechanism

A

AR

fibrin build up in mucosa (plasminogen –> plasmin –> fibrin degradation)

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8
Q

plaques in conjunctival mucosa in plasminogen deficiency

A

ligneous conjunctivitis

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9
Q

ligneous conjunctivitis what and where

A

plaques in conjunctival mucosa in plasminogen deficiency

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10
Q

confirmation test for plasminogen deficienct

A

Fraser-Lendrum stain of ligenous conjunctivities plaques in conjunctival mucosa

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11
Q

Fraser-Lendrum

A

stain of ligenous conjunctivities plaques in conjunctival mucosa
confirmation test for plasminogen deficienct

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12
Q

sickle cell anemia genetic underpinnings

and process generally

A

T–> A substitution
codes valine instead of glutamic acid

chronic hemolutic anemia

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13
Q

sickle cell trait (vs dz)

A

trait – one gene affected

dz – both genes mutated

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14
Q

sickle cell crisis presentation and precipitating factors

A

pain and fever

precipitated by hypoxia, infection, dehydration

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15
Q

acute chest syndrome

A

lung involvement in sickle cell anemia

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16
Q

lung involvement in sickle cell anemia

A

acute chest syndrome

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17
Q

thalassemia what kind of process

A

hypochromic, microcytic anemia

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18
Q

thalassemias differences and grades

A

beta:
minor - one gene
major – two genes, Coley’s anemia
chipmunk facies

alpha:
one gene -- no dz
two genes - trait
three genes
HbH disease
four genes hydrops fetalis
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19
Q

hydrops fetalis and thalassemia

A

alpha with 4 genes affected

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20
Q

HbH disease

A

alpha thalassemia 3 genes

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21
Q

coley’s anemia

A

beta thalassemia, major, two genes

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22
Q

chipmunk face

A

beta thalassemia

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23
Q

excess iron in tissues

A

hemochromatosis

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24
Q

aplastic anemia

A

failure to produce all types of blood cells

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25
Q

failure to produce all types of blood cells

A

aplastic anemia

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26
Q

aplastic anemia associated with

A

Fanconi anemia and dyskeratosis congenita

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27
Q

dyskeratosis congenita heme implications

A

aplastic anemia, Fanconi anemia

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28
Q

people in certain regions are healthy but low on neutrophils

A

benign ethnic neutropenia

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29
Q

genetic neutropenia demographic and syndromes

A 
infants
dyskeratosis congenita, 
cartilage hair synrdomee
Schwachman-Diamond syndrome
severe congenital neutropenia
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30
Q

acquired neutropenia demographic and dzz

A

adults

leukemia, Gaucher dz, osteopetrosis, drugs, vit B12 def, hepatitis

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31
Q

agranulocytosis most affected cells

A

neutrophils

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32
Q

kostmann sydndrome underlying mechanism

A

reduced granulocyte colony stimulating factor (G-CSF)

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33
Q

reduced granulocyte colony stimulating factor (G-CSF) sybdrome

A

kostmann syndrome

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34
Q

cyclic neutropenia genetics

A

neutrophil elastase (ELA2) gene mutation

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35
Q

neutrophil elastase (ELA2) gene mutation

A

cyclic neutropenia

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36
Q

thrombocytopenia 2 possible mechanisms

A

reduced platelets:

reduced production or increased destruction

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37
Q

thrombotic thrombocytopenia purpura

A

increased use of plts in abnormal clot formation

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38
Q

increased use of plts in abnormal clot formation

A

thrombotic thrombocytopenia purpura

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39
Q

idiopathic thrombocytopenia purpura

A

childhood after viral infx, resolves spontaneously

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40
Q

thrombocytopenia in children after viral infx

A

idiopathic thrombocytopenia purpura

resolves spontaneously

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41
Q

polycythemia vera what is and genetics

A

increase in mass of RBC

JAK2 mutation

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42
Q

JAK2 mutation in heme

A

polycythemia vera

increase in mass of RBS

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43
Q

painful burning with erythema of hand and feet in polycythemia vera

A

erythromelalgia

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44
Q

erythromelalgia

A

painful burning with erythema of hand and feet in polycythemia vera

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45
Q

syndromes with higher risk of leukemia

A

down, NF1, Klinefelter, Fanconi anemia

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46
Q

philadelphia chromosome which and where and gene product

A

chronic myeloid leukemia (22q) translocation

bcr-abl gene

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47
Q

CML genetics

A

(22q) translocation – Philadelphia chromosome

bcr-abl gene

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48
Q

bcr-abl gene

A

CML

(22q) translocation – Philadelphia chromosome

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49
Q

22q translocation in hemepath

A

chronic myeloid leukemia (22q) translocation (philadelphia)

bcr-abl gene

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50
Q

tumor like growth from leukemic infiltrate in soft tissues

A

granulocytic sarcoma or extramedullary myeloid tumor or chloroma

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51
Q

chloroma

A

tumor like growth from leukemic infiltrate in soft tissues

aka granulocytic sarcoma or extramedullary myeloid tumor

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52
Q

extramedullary myeloid tumor

A

tumor like growth from leukemic infiltrate in soft tissues

aka granulocytic sarcoma or chloroma

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53
Q

histiocytosis X what is and possible presentations

A

eosinophilic granuloma of bone
chronic disseminated histiocytosis: Hand-Schuler-Christian dz
acute disseminated histiocytosis: Leferer-Siwe disease

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54
Q

chronic disseminated histiocytosis

A

Hand-Schuler-Christian dz
eosinophilic granuloma of bone
histiocytosis X

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55
Q

Hand-Schuler-Christian dz

A

chronic disseminated histiocytosis
eosinophilic granuloma of bone
histiocytosis X

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56
Q

eosinophilic granuloma of bone

A

histiocytosis X
chronic disseminated histiocytosis: Hand-Schuler-Christian dz
acute disseminated histiocytosis: Leferer-Siwe disease

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57
Q

acute disseminated histiocytosis:

A

Leferer-Siwe disease
eosinophilic granuloma of bone
histiocytosis X

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58
Q

Leferer-Siwe disease

A

acute disseminated histiocytosis
eosinophilic granuloma of bone
histiocytosis X

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59
Q

histiocytosis X IHC

A

CD1a or CD207 (+++)

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60
Q

non hodgkin lymphoma higher prevalence in what conditions

A

immune problems:

AIDS, Sjogrens, SLE, RA, organ transplant, Bloom syndrome

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61
Q

mycosis fungoides what is and what from

A

cutaneous T cell lymphoma

from CD4+ T helper lymphocytes

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62
Q

cutaneous T cell lymphoma

aka and what from

A

mycosis fungoides

from CD4+ T helper lymphocytes

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63
Q

stages of mycosis fungoides

A

eczematous, plaque, tumor

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64
Q

sezary syndrome

A

aggressive mycosis fungoides that is really a T cell leukemia affecting multiple organs

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65
Q

aggressive mycosis fungoides that is really a T cell leukemia affecting multiple organs

A

sezary syndrome

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66
Q

sezary cells

A

atypical lymphocytes

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67
Q

lymphocytes in epithelium in hemepath

A

Pautrier’s microabscesses

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68
Q

burkitt’s lymphoma genetics

A

8:14 translocation

c-myc overexpression

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69
Q

8:14 translocation

A

burkitt’s lymphoma

c-myc overexpression

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70
Q

c-myc overexpression

A

burkitt’s lymphoma

8:14 translocation

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71
Q

bence jones proteins

A

light chain proteins produced in multiple myeloma –> renal falure

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72
Q

renal failure in multiple myeloma why

A

bence jones proteins (light chain)

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73
Q

most common site for plasmacytoma

A

spine

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74
Q

acute vs chronic LAD

A

acute: tender, soft, freely movable
chronic: non tender, rubbery, freely movable – ddx from lymphoma which progresses

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75
Q

what tonsil look would be concerning

A

asymmetrical enlargement

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76
Q

phagocytized material in m/ph c/pl

A

tingible bodies

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77
Q

tingible bodies

A

phagocytized material in m/ph c/pl

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78
Q

most common hemophilia, what’s wrong , labs

A

A – f VIII defcy (85% cases)

increased PTT

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79
Q

hemophilia B whats wrong, labs

A

aka christmas dz
f IX defcy
increased PTT

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80
Q

hemophilia vs vWF dz

A

hemophilia PTT increase

vWF PTT and BT increase

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81
Q

most common inherited bleeding disorder

A

vWF disease

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82
Q

common initial sign of hemophilia

A

failure of normal hemostasis after circumcision

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83
Q

infants with hemophilia oral signs

A

oral laceration and ecchymoses on lips and tongue

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84
Q

how much factor VIII is enough for normal function

A

> 25% normal
<5% mild symptoms
severe <1%

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85
Q

deep hemorrhage in hemophilia where, name, consequences

A

hemarthrosis
in mm, ST, joints (kness) –> deformity of joints

–> psedotumor of hemophilia

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86
Q

which coag pathway PTT shows

A

intrinsic

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87
Q

prothrombin function and pathwat

A

extrinsic

measures presence/absence of clotting factors I, II, V, VII, X

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88
Q

plasminogen inheritance

A

AR but more common in F

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89
Q

aka for plasminogen deficiency

A

ligneous conjunctivitis

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90
Q

what does ligneous mean

A

wood-like

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91
Q

oral plasminogen deficiency

A

patchy ulcerated papules and nodules on gingiva; wax and wane

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92
Q

plasminogen deficiency histo

A

acellular eosinophilic material
congo red negative
fraser lendrum confirms it’s fibrin

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93
Q

plasminogen deficiency mucosal surfaces involved

A

conjunctival mucosa (upper eyelid), oral, laryngeal (hoarseness), vaginal

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94
Q

anemia first physical sign

A

paleness of mucous membranes (esp palpebral conjunctiva)

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95
Q

sickle cell anemia mechanis,

A

T-A substitution
valine coded instead of glutamic acid in beta globin chain of hemoglobin

hemoglobin molecule is then prone to aggregation and become rigid and curved like a sickle

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96
Q

sickle cell trait presentation

A

only one gene affected, 40-50% of Hb abnormal, no manifestations

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97
Q

protective effects of sickle cell

A

against malaria

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98
Q

sickle cell dz vs trait

A

dz bpth genes mutated

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99
Q

consequences of sickle cell anemia

A

RBCs block capillaries –> chronic hemolytic anemia, ischemia, infarction

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100
Q

precipitating factors for sickle cell crisis

how presents, what’s affected, how long

A

hypoxia, infection, hypothermia, dehydration

pain and fever; extreme pain from infarction of bones, lung, abdomen
lasts 3-10 days

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101
Q

acute chest syndrome

A

lung involvement in sickle cell crisis 2/2 fat embolism or pneumonia

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102
Q

susceptibility with sickle cell

A

S pneumoniae

most common cause of death in children

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103
Q

sickle cell anemia on dental xrays

A

rarefaction of md and mx, enlarged marrow spaces

coarse stepladder bone trabeculae (bone marrow hyperplasia and increased hematopoiesis)

hair-on-end skull raadiograph

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104
Q

hair-on-end skull raadiograph in a kid

A

sickle cell and thalassemia

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105
Q

reduced trabecular pattern in a kid

A

sickle cell and thalassemia

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106
Q

thalassemia what kind of anemia

A

hypochrmoic, microcytic 2/2 hemolysis of abnormal RBCs

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107
Q

minor beta thalassemia manifest

A

one gene affected, no manifestations

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108
Q

major beta thalassemia aka, manifest

A

Cooley’s or mediterranean anemia
two genes affected
growth of mx/md (chipmunk facies)
bone marrow hyperplasia (increased hematopoiesis with reduced trabecular pattern_, hepatosplenomegaly, hair on end

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109
Q

alpha thalassemia grades of intensity

A

one gene no disease
two genes trait (mild anemia)
three genes - HbH disease (hemolytic anemia and splenomegaly)
four genes - hydrops fetalis (generalized fetal edema)

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110
Q

hydrops fetalis what is and why

A

generalized fetal edema

four gene alpha thalassemia

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111
Q

HbH dz what is and why

A

hemolytic anemia and splenomegaly

three gene alpha thalassemia

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112
Q

two gene alpha thalassemia how look

A

mild anemia

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113
Q

thalassemia therapy complication

A

hemochromatosis – buildup of iron after repeated transfusions

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114
Q

aplastic anemia what is and why

A

BM fails to produce all types of blood cells

autoimmune pancytopenia

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115
Q

triggers of aplastic anemia

A

toxins (benzene), drugs (chloramphenicol), infection (hepatitis)

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116
Q

syndromes with increased frequency of aplastic anemia

A

Fanconi anemia and dyskeratosis congenita

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117
Q

aplastic anemia physical look

A

RBC def-cy –> pallor, tiredness
plt def –> bleeding
WBC def –> most significant; infections

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118
Q

oral look of aplastic anemia

A

petechiae, ecchymoses, pale mucosa, oral ulcerations, gingival hyperplasia

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119
Q

aplastic anemia histo

A

acellular marrow

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120
Q

accelular marrow on histo

A

aplastic anemia

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121
Q

neutropenia labs and consequences

A

n/ph below 1500/mm3
normal 2500-2600
increased susceptibility to infx

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122
Q

genetic neutropenia age and dzz

A

infants

dyskeratosis congenita, cartilage-hair syndrome, schwachamn-diamond syndrome, severe congenital neutropenia

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123
Q

acquired neutropenia age and dzz

A

adults
leukemia, Gaucher, osteopetrosis, drugs (chemo, abx), vit B12 def, infections (He p a and b, HIV, measles, varicella, rubella, TB, typhoid)

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124
Q

neutropenia and infections (especially which); manifest as

A

bacterial infx (esp S aureus)’ abscess formation reduced

middle ear, perirectal and oral infections (ulcerations on gingival mucosa)

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125
Q

cyclic neutropenia genetics, presentation, oral, diagnostic criteria

A

neutrophil elastase (ELA2) gene mutation
18-21 day cycle signs/symptoms
oral: mucosal ulcerations (most severe in gingiva); +/- bone less

dx: sequential CBC (2x/week for 8 weeks) – must be <500 mm3 (3-5 days, 3 cycles)

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126
Q

agranulocytosis which ones are particularly absent

A

n/ph

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127
Q

kostmann syndrome

A

congenital agranulocytosis, low granulocyte colony stimulating factor

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128
Q

congenital agranulocytosis, low granulocyte colony stimulating factor

A

kostmann syndrome

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129
Q

kostmann syndrome presentation

A

ulcers of oral mucosa
gingivitis and gingival necrosis (NUG-like)
periodontitis
decreased hematocrit

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130
Q

oral ulcers, gingivitis/periodontitis, decreased hematocrit

A

kostmann syndrome

congenital agranulocytosis, low granulocyte colony stimulating factor

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131
Q

normal plts and at which count signs of trombocytopenia show

A

normal 200k-400k

signs at <100k/mm3

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132
Q

causes of thrombocytopenia

A

reduced production )BM cancer, CT)
increased destruction (drugs, most common heparin)
sequestration in spleen (gaucher dz, portal HTN)

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133
Q

increased destruction of plts which conditions

A

SLE and HIV

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134
Q

idiopathic (immune) thrombocytopenic purpura

who, why, how long

A

childhood after viral infecion

resolves spontaneously in 4-6 wks (max 6 mos)

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135
Q

tx for childhood purpura

A

idiopathic (immune) thrombocytopenic purpura

resolves spontaneously in 4-6 wks (max 6 mos)

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136
Q

purpura after viral infection

A

idiopathic (immune) thrombocytopenic purpura

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137
Q

abnormal clot formation and increased consumpion of plts name and consequences

A

thrombotic thrombocytopenic purpura

numerous thrombi in small vessels

138
Q

thrombocytopenia histo

A

gingival biopsy

30-40% show fibrin deposits (PAS+) in small vessels –> occlusion

139
Q

polycythemia vera aka and what is

A

primary acquired erythrocytosis
increase in mass of RBC
uncontrolled production of plts and granulocytes also

140
Q

polycythemia vera genetics

A

JAK2 mutation

141
Q

skin complaints polycythemia vera

A

generalized itching after bath without rash

erythromelalgia: painful burning with erythema and warmth of hand and feet

142
Q

oral and systemic complaints in polycythemia vera

A

gingival hemorrhage and myocardial infarction

143
Q

chronic granulomatous disease of childhood aka and what i s

A

bridges-good syndrome, chronic gramulomatous disorder, Quie sundrpme

NADPH oxidase enzyme defect – PMNs can’t destroy bacteria

144
Q

chronic granulomatous disease of childhood what happens and how diagnose

A

gramulomata form in many organs
chronic oral ulcerative lesions

dx: nitroblue-tetrazolium test

145
Q

nitroblue-tetrazolium test for what

A

chronic granulomatous disease of childhood

NADPH oxidase enzyme defect – PMNs can’t destroy bacteria

146
Q

chronic granulomatous disease of childhood msot common infections

A

pneumonia, abscesses, suppurative arthritis, osteomyelitis, bacteremia/fungemia, superficial skin infections (cellulitis or impetigo)

147
Q

child with chronic oral ulcers and multiple abscesses suspect what and how test

A

chronic gramulomatous disease

NADPH oxidase enzyme defect – PMNs can’t destroy bacteria

dx: nitroblue-tetrazolium test

148
Q

leukemia associated with which conditions

A

Down, Bloom, NF1, Schwachman-Diamond, ataxia-telangietasia, klinefelter, Fanconi anemia, Wiskott-Aldrich

149
Q

environmental risks for leukemia

A

chemicals, radiation, viruses (HTLV_1)

150
Q

acute myeloid leukemia age range and survival

A

broad range

40% survival

151
Q

acute lymphoblastic leukemia age range and survival

A

children, 80% survival

152
Q

chronic myeloid leukemia age range and survival

A

adults 0 80% survival

153
Q

chronic lymphocytic leukemia age range and survival

A

older adults; incurable but variable course

154
Q

philadelphia chromosome what, which dz, prodycts

A

CML
22;9
bcr-abl gene and a chimeric protein with tyrosine kinase activity

155
Q

which heme malignancy produces a chimeric tyrosine kinase

A

CML
22;9 – philadelphia chromosome
bcr-abl gene and a chimeric protein with tyrosine kinase activity

156
Q

22;9 chrom

A

CML
philadelphia
bcr-abl
tyrosine kinase

157
Q

early stage AML name

A

myelodysplasia syndromes

158
Q

leukemia oral look

A

ulcerations (esp gingival – depp, punched out), candidiasis, herpes

159
Q

chloroma what is and where most common (site and condition)

A

tumor like growth from leukemic infiltrate in soft tissues

gingival infiltration most seen in AML

160
Q

blast transformation where and what happens

A

in CML

cells become less differentiated, proliferate and –> death in 3-6 months

161
Q

induction chemo vs maintenance chemo

A

induction: high doses to destroy cancer cells –> remission

maintenance – low –> maintain remission

162
Q

richter syndrome

A

CLL becoming large cell lymphoma

163
Q

CLL becoming large cell lymphoma

A

richter syndrome

164
Q

tumor like growth from leukemic infiltrate in soft tissues

A

chloroma

gingival infiltration most seen in AML

165
Q

leukemia with the most gingival infiltration

A

AML

166
Q

forms of langerhans cell histiocytosis

A

eosinophilic granuloma of bone (mono or polyostotic)
chronic disseminated histiocytosis (Hand-Schuler-Chritian dz)
acute disseminated histiocytosis (Leferer-Siwe)

167
Q

hand-schuler-christian dz other name and signs

A

chronic disseminated histiocytosus/LCH

bone lesions, exophthalmos, diabetes insipidus

168
Q

LCH xray

A

punched out RL, scooped out, teeth floating in air, most in post MD

169
Q

punched out RL, scooped out, teeth floating in air, most in post MD

A

LCH

170
Q

adult LCH ddx

A

can mimic severe localized periodontal disease in adult

171
Q

birbeck granules

A

rod shaped structures in c/pl of langerhans cells on EM

172
Q

rod shaped structures in c/pl of langerhans cells on EM

A

birbeck granules

173
Q

langerhans cell histiocytosis IHC

A 
CD1a or CD207 (+++)
peanut agglutinin (PNA)
174
Q

microorganism with hodgkins lymphoma

A

EBV

175
Q

age and location for hodgkin

A

75% in cervical and supraclavicular LNs

15-35 and 50+

176
Q

typical presentation of hodgkin lymphoma

A

nontender LAD – progresses from one LN chain to another

weight loss, fever, night sweats, pruritus

177
Q

histo types of hodgkin lymphoma

A

nodular lymphocyte predominant (popcorn cells, good prognosis)
classical (lymphocyte rich; nodular sclerosis - 80%, more in F, 2nd decade, Reed Sternberg, lacunar cells, good prognosis; mixed cellularity - 20%, eosinophils present; lymphocyte depletion - most aggressive; unclassifiable)

178
Q

popcorn cells which malignancy

A

nodular lymphocyte predominant hodgkin lymphoma

good prognosis

179
Q

nodular sclerosis demographic and what is

A

classical subtype of hidgkin lymphome
80% (most common ig)
more in F 2nd decade
Reed Sternbers, lacunar cells, good prognosis

180
Q

eosniphils present in which lymphoma

A

hodgkin classical mixed cellularity

181
Q

most aggressive hidgkin lymphoma

A

classical lymphocyte depleted

182
Q

reed sternberg which malignancy and IHC

A

classicla hodgkin lymphoma
owl eye or pennies on a plate

CD15+ and CD30++

183
Q

treatment scheme for hodgkin

A

ABVD

adriamycin, bleomycin, vinblastine, dacarbazine

184
Q

owl eye cell

A

Reed Sternberg, classical Hodgkin

185
Q

how does non-hodgkin lymphoma grow

A

solid mass

186
Q

nonhodgkin lymphoma predisposing conditions

A

immune problems:

AIDS, Sjohrens, SLE, RA, organ tranplant, Bloom syndrome

187
Q

infectious agents and lymphoms

A

EBV – Hodgkin and Burkitt

H pylori - MALT lymphoma of stomach

188
Q

most common lymphomas of oral

A

Non Hodgkin
usually extranodal
DLBCL (high grade) most common, then follicular

189
Q

most common oral site for lymphoma

A

waldeyers ring, then hard palate

190
Q

most common lymphoma of salivary glands

and histo look

A

follicular

dumbbell shaped, back to back follicles (little mantle)

191
Q

prognostic histo factor in follocular lymphoma

A

rati of centroblasts to centrocytes

192
Q

IHC DLBCL

+ genetics

A

CD5 +/-
CD10 +/-
CD19 +
CD20 +

translocation 3q27 and t(14:18)

193
Q

t(14:18)

A

DLBCL and follicular lymphoma

194
Q

CD5 +/-
CD10 +/-
CD19 +
CD20 +

A

DLBCL

195
Q

IHC follicular lymphoma and genetics

A 
CD5 - 
CD10 +
CD19+
CD20+
t(14;18), bcl2 overexpression
196
Q

bcl2 overexpression

A

follicular lymphoma

197
Q

SLL/CLL IHC

A

CD5 +
CD19 +
CD20+
CD23 +

198
Q

CD5 +
CD19 +
CD20+
CD23 +

A

SLL/CLL

199
Q

MCL IHC and genetics

A

CD5+
CD19+
CD20+
CD23 -

t(11;14)

200
Q

CD5+
CD19+
CD20+
CD23 -

A

MCL

201
Q

t(11;14)

A

MCL

202
Q

MALT IHC

A

CD10 -
CD20+
CD21+
CD22 +

203
Q

CD10 -
CD20+
CD21+
CD22 +

A

MALT

204
Q

monoclonality techniques

A

IHC light chains;
gene rearrangement studies
flow cytometry

205
Q

most common peripheral T cell lymphoma

A

angioimmunoblastic T cell lymphoma

aka angioimmunoblastic T cell lymphadenopathy with dysproteinemia

206
Q

angioimmunoblastic T cell lymphoma histo and main ddx

A

main ddx from Hodgkin

polymorphous lymph node infiltrate
marked increase in follicular dendritic cells and high endothelial venules

207
Q

polymorphous lymph node infiltrate

marked increase in follicular dendritic cells and high endothelial venules

A

angioimmunoblastic T cell lymphoma

208
Q

angioimmunoblastic T cell lymphoma signs

A

fever, malaise, joint pain, skin rash

209
Q

mycosis fungoides aka and cells of origin

A

cutaneous t cell lymphoma

from t helper CD4+ lymphocytes

210
Q

stages of mycosis fungoides

A

eczematous (histo v subtle; similar to psoriasis)
plaque (sezary cells in epith)
tumor (sezry cells in dermis and epidermis)

211
Q

oral look of mycosis fungoides

A

erythematous, indurate dplaques or nodules, typically ulcerated

212
Q

sezary syndrome

A

aggressive form of mycosis fungoides

dermatopathic t cell leukemia affecting multiple organs (mostly kidney and liver)

213
Q

aggressive form of mycosis fungoides

A

sezary syndrome

dermatopathic t cell leukemia affecting multiple organs (mostly kidney and liver)

214
Q

sezary cells look and IHC

A

mycosis cells - atypical lymphocytes
cerebriform nucleus – marked infolding of nuclear membrane

IHC CD4+

215
Q

pautrier’s microabscesses

A

aggregates of sezary cells in epithelium

plaque stage of mycosis fungoides

216
Q

aggregates of sezary cells in epithelium

A

pautrier’s microabscesses

plaque stage of mycosis fungoides

217
Q

tx for mycosis fungoides

A

antibody against CD52

218
Q

types of Burkitt lymphoma

A

endemic (African, 95% EBV)
sporadic (American, 25% EBV)
immunodef-cy associated

219
Q

demographic for endemic burkitt

A

boys (peak 7yo)
post mx more
young pts have more jaw involvement

220
Q

early xray sign for burkitts

A

patchy loss of lamina dura

221
Q

burkitt IHC

A

CD10+

Ki67 almost 100%

222
Q

burkitt histo look

A

starry sky

reactive lightly stained histiocytes (tingible m/ph) in a background of dark tumor cells

223
Q

starry sky

A

burkitt

224
Q

burkitt molecular

A

t(8:14)

c-MYC overexpression

225
Q

t(8:14)

A

burkitt

c-myc

226
Q

c-myc

A

burkitt

t(8:14)

227
Q

extranodal NK/T cell lymphoma aka

A

angiocentric t cell lmphoma

228
Q

extranodal NK/T cell lymphoma presentation

A

aggressive destruction of midline structures of palate and nasal fossa
deep necrotic ulcer in midlate palate with oronasal fistula

229
Q

aggressive destruction of midline structures of palate and nasal fossa
deep necrotic ulcer in midlate palate with oronasal fistula

hemepath

A

extranodal NK/T cell lymphoma

230
Q

lymphomatoid granulomatosis associated with and what is

A

extranodal NK/T cell lymphoma

B cell proliferation induced by EBV

231
Q

B cell proliferation induced by EBV

A

lymphomatoid granulomatosis

extranodal NK/T cell lymphoma

232
Q

extranodal NK/T cell lymphoma histo and IHC

A

mixed inflammatory cells around blood vessels (angiocentric
necrosis

CD56 and granzyme B

233
Q

granzyme B stains in what

A

extranodal NK/T cell lymphoma

234
Q

plasmacytoma what is and where

A

unifocal proliferatino of plasma cells

most common site spine

235
Q

plasmacytoma IHC

A

cyclin D1 and CD56+

236
Q

extramedullary plasmacytoma vs classic

A

in soft tissues
90% head and neck
cyclin D1 and CD56 negative

237
Q

plasmacytoma vs multiple myeloma

A

plasmacytoma less protein M
no bone marrow infiltration
no signs of anemia, hypercalcemia, or renal failure

238
Q

plasmacytoma prognosis

A

50% develop MM in 3 years

extramedullary only 30% tho

239
Q

multiple myeloma demographic

A

most common heme malignancy in black ppl

2x common in black

240
Q

most characteristic symptom of multiple myeloma

A

lumbar spine pain

241
Q

cause of metastatic calcification in MM

A

hypercalcemia 2/2 tymor osteolysis

242
Q

bence jones proteins what is and where

A

excess light chain protein (k or l) produced by MM cells
urine of 30-50% pts
can cause renal failure

243
Q

characteristic deposits in oral cavity in MM patients

A

amyloid – oral cavity and periorbital

15% MM patients

244
Q

MM IHC

A

CD138, k and l to show monoclonality

245
Q

monoclonal gammopathy

A

excess immunoglobulin (M protein) in serum and urine

246
Q

MM what marks worse prognosis

A

high β2 microglobulin, low albumin, older pts

247
Q

castleman’s disease aka

A

angiofollicular lymph node hyperplasia

248
Q

castleman disease what is

A

solitary or multicentric benign growth of lymphoid tissue in young pts (<30yo)

249
Q

castleman disease histo look

A

node architecture obliteration with scattered depleted small follicaulr centers surrounded by a cuff of small lymphocytes (onion skin) divided by concentric rings of reticulin fibers resembling Hassall’s corpuscles and penetrated by hyalinized vessels

250
Q

node architecture obliteration with scattered depleted small follicaulr centers surrounded by a cuff of small lymphocytes (onion skin)

A

castleman disease

251
Q

castleman disease systemic form clinical and syndromic association

A 
adenopathy, fever, anemia, elevated ESR and hypergammaglobulinemia
older patietns with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes)
poor prognosis (assoc w lymphoma)
252
Q

cause of castleman dz

A

HHV8

253
Q

Kikuchi Fujimoto dz aka and what is

A

histiocytic necrotizing lymphadenitis

self limiting benign inflamm condition affecting cervical lymp nodes

254
Q

kikuchi fujimotot demographic

A

asians, F>M

255
Q

Kikuchi Fujimoto presentation

A

fever and leukopenia

256
Q

Kikuchi Fujimoto histo and patterns

A

three patterns: proliferative, necrotic, xanthomatous

necrosis surrounded by histiocytes, lymphocytes, plasma cells
no neutrophils

257
Q

rosai dorfmann dz aka and where

A

sinus histiocytosis with massive lymphadenopathy
90% in cervical LN

resolves spontaneously

258
Q

rosai dorfmann what predisposes

A

pts with autoimmune lympoproliferative syndrome with Fas mutation

259
Q

emperipolesis what condition and what is

A

rosai dorfmann dz
lymphocytophagocytosis
lymphocytes inside histiocytes
often forming wreath-like rings within cytoplasm

plasma cells, n/ph, RBC may be found inside histiocytes

also seen in cutaneous t cell lymphoma

260
Q

rosai dorfmann IHC

A

S100+, CD68+

CD1a neg

261
Q

kawasaki dz aka

A

mucocutaneous lymph node syndrome

acute febrile vasculitic syndrome of early childhood

262
Q

kawasaki HLA

A

BW22

263
Q

leading cause of acquired heart dz in children

A

kawasaki dz

also risk factor for adult ischemic heart dz

264
Q

kawasaki presentation

A

high fever 1-2 weeks
bilateral congestion of ocular conjunctivae
dryness, redness, fissuring of lips
diffuse reddening of oral and pharyngeal mucosa
LAD (necrotic lymph node)
oral – strawberry tongue

265
Q

high fever 1-2 weeks
bilateral congestion of ocular conjunctivae
dryness, redness, fissuring of lips
diffuse reddening of oral and pharyngeal mucosa
LAD (necrotic lymph node)
oral – strawberry tongue

A

kawasaki

266
Q

angiolymphoid hyperplasia with eosiophilia aka

A

epithelioud hemangioma

267
Q

angiolymphoid hyperplasia with eosiophilia demographic

A

frequent in H&N of young asian men

can affect both sexes

268
Q

angiolymphoid hyperplasia with eosiophilia presentation and mechanism

A

proliferation of small vessels lined by plump endothelial cells
report trauma, pulsatile sensation, pruritus
usually more superficial (dermis) –> multiple papules and nodules
other sites: upper lip, salivary glands, lungs, bone

269
Q

kimura’s dz demographic

A

h&n of young adult asian men (strong racial predilection)

270
Q

kimura dz similar to what but how different

A

similar to angiolymphoid hyperplasia with eosiophilia

kimura deeper – subqt tissue, salivary glands, LN

fever, malaise, LAD (ALHE has no symptoms)

endothelial cells not very large and flatter (ALHE is more prominent vascular condition)

can show germinal centers (rare in ALHE)

271
Q

kimura dz labs

A

peripheral blood eosinophilia and elevated IgE

272
Q

mutation in sickle cell

A

thymine (glutamic acid) for adenine (valine) in DNA in beta-globin chain

273
Q

step ladder differential

A

sickle cell or myxoma; depends on extent

274
Q

which dzz provide resistance to malaria

A

thalassemia, sickle cell

275
Q

under how many neuts pulmonary infection

A

<500

276
Q

hemophilia bleeding test

A

PTT increased

277
Q

vWF dz bleeding test

A

PTT, PFA/bleeding time increased

278
Q

warfarin bleeding test

A

PT increased

279
Q

aspirin bleeding test

A

bleeding time/PFA increased

280
Q

PT factors

A

1, 2, 5, 7, 10

281
Q

PTT factors

A

2, 5, 8-12

282
Q

diagnosis of cyclic neutropenia

A

<500 for at least 3 successive cycles

283
Q

thrombocytopenia when first evident

A

<100,000

284
Q

thrombocytopenia when severe

A

<10,000

285
Q

ADAMTS3 function

A

cleaves vWF

deficient in thrombotic thrombocytopenic purpura

286
Q

immune thrombocytopenic purpura acute vs chronic

A

Acute : after a viral infection in kids, resolves within 4-6 wks
 Chronic: woman 20-40 yrs

287
Q

early stages of AML

A

myelodysplasia syndromes

288
Q

myelodysplasia syndromes

A

early stages of AML

289
Q

reduction in number of RBC and WBC due to crowding of BM by malignant cells

A

Myelophthisic anemia:

290
Q

Myelophthisic anemia:

A

reduction in number of RBC and WBC due to crowding of BM by malignant cells

291
Q

tumor like keukemia

A

chloroma/granulocytic sarcoma/myeloid sarcoma

292
Q

imatinib

A

tyrosine kinase inhibitor for CML

293
Q

2nd gen tyrosine kinase inhibitor

A

imatinim first

nilotinib 2nd

294
Q

monoclonal antobody to CD20

A

rituximab

295
Q

rituximab

A

monoclonal antobody to CD20

296
Q

chronic LCH triad

A

diabetes insipidus 2/2 kidney low ADH
exophthalmos
bone lesions

297
Q

5 types of classic hodgkin

A 
Lymphocytic rich (6%)
o Nodular sclerosis (60%)
o Mixed cellularity (15-30%)
o Lymphocyte depletion (1%)
o Non classifiable
298
Q

hidgkin that’s not classic

A

nodule lymphocyte predominant

299
Q

lymphoma treatment regimens

A

ABVD: Adrimycin, Bleomycin, Vinblastine, Dacarbazine ( used mostly )
 MOPP: Mechlorethamine, Oncovine, Procarbazine, Prednisone ( not used anymore due
complications )

300
Q

Mycosis Fungoides what is actually

A

(cutaneous T-cell Lymphoma)

301
Q

stages of mycosis fungoides

A

Eczematous, Plaque, Tumor stage:

302
Q

special tropic feature of mycosis fungoides

A

Epidermotropism: propensity to invade the epidermis of the skin

303
Q

aggressive form of Mycosis fungoides

A

Sezary syndrome:

304
Q

atypical cells in mycosis fungoides

A

Sezary cells or mycosis cells

305
Q

aggregation of lymphocytes in mycosis fungoides

A

Pautrier microabscesses:

306
Q

nuclei of lymphocytes in mycosis fungoides

A

Cerebriform nucleus: Abnormal nucleus ( folding of nuclear membrane)

307
Q

Pautrier microabscesses which stage

A

mycosis fungoides plaque stage

308
Q

treatment for mycosis fungoides

A

Photochemotherapy PUVA
Monoclonal Antibody CD52
Denileukin diftitix : diphtheria toxin targets IL-2 receptors
Extracorpeal photopheresis ingestion of 8-methoxypsoralen, irradiate WBC outside body

309
Q

EBV related processes (6)

A 
Mono
Oral Hairy Leukoplakia
Burkitt Lymphoma
Nasopharyngeal Carcinoma
Lymphoepithelial carcinoma
Hepatocellular carcinoma
310
Q

burkitt genetics

A

t(8:14) (q24;q32)

c-myc

311
Q

t(8:14)

A

burkitt , c myc

312
Q

Ki67 in burkitt

A

almost 100%

313
Q

classic histo look of burkitt

A

starry sky 2/2 histiocytes with abundant c/pl

night sky is the dark staining lymphoma cells

314
Q

types of burkitt

A

African-EBV related in jaws of children

  1. Endemic
  2. Sporadic-American and abdominal mass
  3. HIV/Immunodeficiency
315
Q

tx for burkitt

A

Treatment: High dose Cyclophosphamide chemotherapy an alkylating agent of the
nitrogen mustard type (specifically, the oxazaphosphorine group)

316
Q

polycythemia genetics

A

JAK 2

317
Q

JAK 2

A

polycythemia

318
Q

CML genetics

A

22+9 /BCR-ABL

319
Q

BCR-ABL

A

CML 22+9

320
Q

22+9

A

CML BCR-ABL

321
Q

cyclic neutropenia genetics

A

ELA-2 /ELANE

322
Q

ELA-2

A

cyclic neutropenia

323
Q

TTP genetics

A

ADAMTS13

324
Q

ADAMTS13

A

TTP

325
Q

most chatacteristic presenting signs (2) in multiple myeloma

A

lumbar pain and kidney 2/2 bence jones light chains

326
Q

how many multiple myeloma pts get amyloid

A

15%

327
Q

amyloid stains

A

congo red, crystal violet

328
Q

primary amyloidosis amyloid

A

AL

329
Q

multiple myeloma amyloid

A

AL

330
Q

secondary amyloidosis amyloid

A

AA

331
Q

hemodialysis amyloid

A

Aβ2 M

332
Q

AL amyloid which conditions

A

primary amyloidosis and MM

333
Q

AA amyloid which condition

A

secondary

334
Q

Aβ2 M amyloid which condition

A

hemodialysis

335
Q

IHC exytamedullary plasmacytoma vs MM

A

weak or negative for cyclin D1 and CD56

336
Q

radiation dose for plasmacytoma

A

at least 40Gy

337
Q

iron deficiency anemia sybdrome

A

Plummer Vinson

338
Q

pernicious anemia aka and causes

A 
aka megaloblastic
decreased B12 (cobalamin) and folic acid
339
Q

microcytic anemia criteria and conditions

A

MCV ≤ 80fl

TAILS
Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning
Siderblastic anemia
340
Q

normocytic anemia criteria and conditions

A

MCV 80-100 fl

TTP
Hemolysis
Osteopetrosis
Sickle cell
Pregnancy
Many other examples
341
Q

macrocytic anemia criteria and conditions

A

MCV ≥100 fl

Megaloblastic anemia
(B12 or foliate deficiency)
Reticulocytosis
Hemolytic anemia
Chemo

Others : a few examples
Alcohol, Liver disease
Hypothyroidism, pregnancy
Aplastic anemia, MM,

OP - Yield (19 decks)

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