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Paige: Heme/Onc Module! > Clin Med Diseases (RBC/platelets) > Flashcards

Clin Med Diseases (RBC/platelets) Flashcards

1
Q

Thrombocytopenia is…

A

low platelets

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2
Q

What might cause thrombocytopenia?

A
  1. impaired bone marrow production
  2. increased destruction of platelets
  3. splenic sequestration

MC cause: bleeding

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3
Q

3 types of thrombocytopenia

A

idiopathic

thrombolic

von willebrand

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4
Q

What is the pathophysiology of idiopathic thrombocytopenia purpura?

A

the immune system destroys platelets

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5
Q

When does idiopathic thrombocytopenia purpura most often occur (etiology)?

A

immune response:

after infection, with HIV, with SLE

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6
Q

Who would most likely get idiopathic thrombocytopenia purpura (ITP)?

A

Acute: self-limited, autoimmune IgG, children (viral)

Chronic: coexisting with other autoimmune, any age

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7
Q

What is the clinical presentation of idiopathic thrombocytopenia purpura (ITP)?

A

mostly asymptomatic

may have sudden onset of bruises, petechiae, mucosal bleeding

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8
Q

How would you diagnose idiopathic thrombocytopenia purpura (ITP)?

What will your lab values be?

A

Acute: CBC shows decreased platelets (10K-20K), eosinophilia, mild lymphocytosis

Chronic: platelet count 25K - 75K

Peripheral smear shows megakaryocytes

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9
Q

How do you treat idiopathic thrombocytopenia purpura (ITP)?

A

Acute: self-limiting, sometimes corticosteroids, IVIg, splenectomy

Chronic: REFER.

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10
Q

What is the pathophysiology of thrombolic thrombocytopenia purpura (TTP?)

A

platelets aggregate in microcirculation, resulting in a clot, then a bleed

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11
Q

How is thrombolic thrombocytopenia purpura (TTP) classified?

A

Microangio-pathic hemolytic anemia

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12
Q

Who gets thrombolic thrombocytopenia purpura (TTP?) What is the epidemiology?

A

RARE but fatal. Found in previously healthy people.

20-50 years of age, female, pregnant, estrogen use, HIV/AIDS, certain medications

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13
Q

How does TTP present clinically?

A

purpura (big bruise), petechiae, bruising

neurologic abnormalities

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14
Q

How do you diagnose TTP? What will lab values be?

A

CBC: anemia, schistocytes, î LDH, î indirect bilirubin, î D-dimer

Ab against ADAMTS 13

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15
Q

How do you treat TTP?

A

Refer!!!

emergency plasma transfusion

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16
Q

What is the pathophysiology of von Willebrand disease (vWd)?

A

von Willebrand protein helps adhere platelets to vessels, helps stabilize factor VIII;

in vWd, the body is deficient in vW protein;

therefore, vWd affects platelet aggregation and prolongs bleeding time

The most common hereditary coagulation disorder

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17
Q

How do you classify vWd? (3 types)

A

type 1: most common with mild bleeding

type 2: more severe than 1

type 3: rare, most severe

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18
Q

Who gets vWd and how?

A

autosomal dominant, congenital

both men and women

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19
Q

What is the clinical presentation of vWd?

A

bleeding in nasal, sinus, vaginal, and GI mucus membranes

menorrhagia

bleeding exacerbated by NSAIDs, aspirin

bleeding relieved by estrogen, pregnancy

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20
Q

How do you diagnose vWd? Lab values?

A

PT normal, but PTT mildly prolonged

PFA prolonged

vWF low

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21
Q

How do you treat vWd?

A

Desmopressin (DDAVP) - to stim. endothelial release of vWF

Factor VIII concentrates to replace Factor VIII

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22
Q

Hemophilia A pathophys

A

deficient in Factor VIII

therefore, impaired coagulation pathway/can’t make platelets

leads to excess bleeding

Most severe bleeding d/o

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23
Q

How do people get Hemophilia A? Who gets it?

A

X-linked recessive

only in males

2nd most common congenital coagulopathy

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24
Q

What are the symptoms of Hemophilia A?

A

excessive bruising

repeated bleeding episodes with hemarthrosis, epistaxis, intracranial bleeds

blood in vomit, stool, urine, soft tissue, and gingiva

excessive bleeding after surgery/trauma/compartment syndrome

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25
Q

How do you diagnose Hemophilia A?

A

PTT prolonged

PT, PFA, fibrinogen level, platelets NORMAL

dec. Factor VIII, C levels

vWF is normal

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26
Q

What is the tx for hemophilia A?

A

infuse pt with recombinant Factor VIII concentrate*

Desmopressin (DDAVP) to elevate Factor VIII

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27
Q

What should patients with Hemophilia A avoid?

A

aspirin!

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28
Q

What is the pathophys of Hemophilia B?

(AKA Christmas disease)

A

deficiency of Factor IX

therefore, also excess bleeding

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29
Q

Who gets Hemophilia B and how?

A

X-linked recessive dz

Males

less common than A

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30
Q

What is the clinical presentation of Hemophilia B?

A

Similar to A!

excessive bleeding, bruising, hematomas, hemarthrosis, compartment syndrome

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31
Q

How do you diagnose Hemophilia B?

A

low Factor IX

prolonged PT

normal platelet count

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32
Q

How do you tx Hemophilia B?

A

Literally do the same thing as Hemophilia A, except use Factor IX.

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33
Q

What is the pathophys of Disseminated intravascular coagulation (DIC)

A

there is activation of intravascular coagulation

there is also increased fibrinolysis

therefore, you get both excess clotting AND bleeding

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34
Q

What causes DIC?

A

clotting cascade triggered by:

surgery, trauma

sepsis, vascular disorders

pregnancy, malignancy

burns, shock

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35
Q

What are the clinical symptoms of DIC?

A

may present as petichiae, purpura, oozing, or severe postpartum bleeding

diffuse bleeding

microvascular thrombosis

bleeding in basically every important organ system

shock

(can lead to organ failure)

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36
Q

What will lab values show you for DIC?

(there is not a huge focus on this anywhere)

A

prolonged PT

prolonged PTT

abnormal PFA/low platelets

decreased fibrinogen

increased fibrin degradation

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37
Q

How do you treat DIC?

A

treat the cause.

can give frozen plasma, platelets, or fibrinogen

can give heparin to dec coagulation

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38
Q

What is the outcome of people with DIC?

A

most die

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39
Q

In patients with clots, will they have high or low D-dimer?

A

high

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40
Q

What is the most common acquired coagulopathy?

A

Vit K deficiency

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41
Q

What causes Vit K deficiency?

A

poor diet, liver failure, malabsorption, malnutrition, some drugs (broad spectrum abx)

42
Q

What are the clinical features of Vit K deficiency?

A

typically post-op

not eating well, receiving broad spectrum abx

maybe soft tissue bleeding

43
Q

Lab findings for Vit K deficiency?

A

PT prolonged

PTT prolonged

Fibrinogen, platelets normal

Levels of Vit K and clotting factors decreased

44
Q

Tx of Vit K deficiency

A

treat underlying cause.

Give Vit K

treat hemorrhage with frozen plasma

Eat a diet high in leafy veggies

45
Q

What causes iron deficiency anemia?

A

Most common

menstruation, pregnancy, bleeding

46
Q

What causes folate deficiency anemia?

A

most likely poor dietary intake (vegan diet)

47
Q

What causes B12 and pernicious anemia?

A

B12 defiency in diet (vegans)

Could lack intrinsic factor (“pernicious disorder”), have ileum disorder

Some drugs (H2 antagonists)

48
Q

What causes aplastic anemia?

A

This is a disorder of pluripotent bone marrow stem cells

Reduction of RBC, WBC, and platelets

Unknown cause

49
Q

What causes hemolytic (G6PD) anemia?

A

X-linked

RBCs break down prematurely, most common enzyme deficiency (G6PD)

50
Q

What causes sickle cell anemia?

A

abnormal Hgb

autosomal recessive

51
Q

What do the cells look like in iron anemia?

A

hypochromic

microcytic

52
Q

What do cells look like in folate anemia?

A

macrocytic

megaloblastic

53
Q

What do the cells look like in pernicious anemia?

A

macrocytic/megalobastic

54
Q

What do the cells look like in aplastic anemia?

A

hypoproliferative

55
Q

What do the cells look like in hemolytic anemia?

A

normocytic

56
Q

What do the cells look like in sickle cell anemia?

A

sideroblastic

sickled under stress

57
Q

What are the risks for iron anemia?

A

pre-menopause

pregnancy

frequent blood donor

diet (milk, Vit C, Zn), family history

58
Q

What are the risks for folate anemia?

A

alcohol

pregnancy

some medications (methotrexate)

59
Q

What are the risks for B12 anemia? (pernicious)

A

being vegan

gastric surgery, pancreatic insufficiency, Chron’s disease

60
Q

What are the risks for aplastic anemia?

A

exposure to toxins via certain meds

Some blood/autoimmune dz

Thymus tumors

61
Q

What are the risks for G6PD deficiency anemia?

A

being AA or Greek

Male

stress (inc hemolysis)

sulfa drugs, fava beans, nitrofurantoin

62
Q

What are the risks for sickle cell anemia?

A

being AA (autosomal recessive)

(many things can trigger a sickle cell crisis)

63
Q

What is the clinical presentation of someone with iron anemia?

A

weakness/fatigue, tachy, exertional dyspnea, pallor

pica, cheilosis, glossitis

may have koilonychia (spoon-shaped nails)

64
Q

What is the clinical presentation of someone with folate anemia?

A

tachy, pallor, exertional dyspnea, fatigue

sore tongue (glossitis)

GI symptoms

65
Q

What is the clinical presentation of someone with pernicious anemia?

A

tachy, pallor, exertional dyspnea, fatigue

GI symptoms/malabsorption

glossitis

neurological symptoms

66
Q

What is the clinical presentation of someone with aplastic anemia?

A

tachy, pallor, exertional dyspnea, fatigue

insidious onset, palpitations, progressive weakness, weight loss

heavy bleeding, systolic ejection murmur, petechiae

fever

Congenital: short, microcephaly, abnormal thumbs, oral leukoplakia

67
Q

What’s the clinical presentation of someone with G6PD anemia?

A

tachy, pallor, exertional dyspnea, fatigue

females usually have NO symptoms

jaundice, dark urine

68
Q

What’s the clinical presentation of someone with sickle cell anemia?

A

severe, recurrent abd pain

acute chest syndrome

splenomegaly, jaundice, fever

pain everywhere

swollen hands and feet (dactylitis)

NO erythema

AVN, bone infarctions, vascular occlusions

69
Q

Diagnostics for iron anemia?

A

MCV low (microcytic)

reticulocytes low (bone marrow problem)

Fe, ferritin low

TIBC high

periph smear: anisocytosis, poikilocytosis

70
Q

Dx for folate anemia?

A

folate low

MCV high

low reticulocyte

LDH high

smear: macro-ocalocytes & hypersegmented polymorphonuclear cells

71
Q

Dx for B12 anemia?

A

B12 low

MCV high

low reticulocyte

Intrinsic factor Ab

72
Q

Dx for aplastic anemia?

A

All blood cells low

reticulocytes low

Fe high

TIBC normal

Get FULL workup (CBC, liver fn, UA,

73
Q

Dx for g6pd anemia?

A

periph smear: Heinz bodies, bite cells

G6PD low

reticulocytes high

bilirubin high

74
Q

Dx for sickle cell anemia?

A

Screen: Sickledex test

Electrophoresis (Hgb S is >50%)

peripheral smear for sickled cells, target cells, nucleated RBCs, Howell-Jolly bodies

reticulocyte high

LDH high

bilirubin high

ferratin high

WBC high

75
Q

Tx for iron anemia?

A

Ferrous sulfate

Find source of bleeding

76
Q

Tx for folate anemia?

A

Folic acid supplement

Avoid alcohol

77
Q

Tx for pernicious anemia?

A

Vit B12 supplement (IM)

78
Q

Tx for aplastic anemia?

A

Stem cell replacement (bone marrow/periph blood transplant)

79
Q

Tx for G6PD anemia?

A

mostly self-limiting

avoid stress

avoid certain medicines and oxidative drugs

80
Q

Tx for sickle cell anemia?

A

Oxygen, IV fluids, transfusions

Children should get LD penicillin daily from birth to 6 years, pneumovax, as well as lots of other screening

81
Q

What is the pathophys of chronic anemia?

A

Chronic infections/inflammation causes increased hepcidin levels, which blocks iron absorption in the gut and blocks iron release from bone marrow

82
Q

What causes chronic anemia?

A

chronic anemia is a sx rather than a disease

autoimmune diseases: SLE, RA

sickness: cancer, hospitalization

83
Q

What do cells look like in chronic anemia?

A

microcytic or normocytic

normochromic

hypoproliferative

84
Q

What are the symptoms of chronic anemia?

A

tachy, pallor, exertional dyspnea, fatigue

chronic inflammation

85
Q

How do you diagnose chronic anemia?

A

Elevated CRP and ESR

Normal-high ferritin level

86
Q

Treatment of chronic anemia?

A

Treat underlying cause

Transfusion if symptomatic

87
Q

What will chronic anemic patients not respond to for treatment?

A

epo or iron supplements

88
Q

What is the pathophysiology of alpha-thalassemia?

A

deficient synthesis of alpha Hgb chain (low Hgb A)

usually leads to RBC hemolysis & increased iron

89
Q

What is the pathophysiology of beta-thalassemia?

A

deficient synthesis of beta-Hgb (low Hb A2 and low Hb F)

usually leads to RBC hemolysis & increased iron

90
Q

What do cells look like in alpha and beta thalassemia?

A

microcytic

hypochromic

91
Q

Who gets alpha and beta thalassemia and how?

A

Alpha - Southeast Asia and Chinese

Beta - African and Mediterranean

Hereditary

92
Q

What are clinical features of alpha-thalassemia?

A

anywhere from silent to profound defecits

symptoms of iron deficiency

in its worst form, can cause stillbirths from hydrops fetalis

93
Q

What is the clinical presentation of B-thalassemia major (Cooley anemia)?

A

anywhere from silent to profound defecits

sx at 4-6 months of age

anemia, growth retardation, abnormal facial structure, pathologic fx, osteopenia, bone deformities, hepatosplenomegaly, jaundice, cardiac issues

94
Q

Dx of alpha thalassemia

A

Hct low

Hgb very low

Electrophoresis shows Hemoglobin H (confirms)

Periph smear shows target cells, poikilocytes

Reticulocytes high

Fe, ferritin normal or high

95
Q

Dx of beta thalassemia

A

Hct very low

Hgb very low

Electrophoresis shows Hgb A2, F

Periph smear shows target cells, poikilocytes, basophilic stippling, nucleated RBCs

Reticulocytes high

Fe, ferratin normal or high

96
Q

Tx of thalassemia

A

If Hgb H (alpha): folic acid supplements; avoid iron and oxidative drugs

If beta: transfusions, but be VERY careful of iron overload; parenternal or oral deferoxamine after; allogeneic bone marrow transplant/splenectomy

97
Q

What is the pathophys of spherocytosis

A

RBCs are shaped like spears

Leads to premature breakdown of RBCs

Genetic

98
Q

Who gets spherocytosis?

A

Northern Europeans

99
Q

What are the symptoms of spherocytosis?

A

fatigue, irritability, SOB, weakness

100
Q

How do you diagnose spherocytosis?

A

periph smear - spherocytes

bilirubin high

CBC for anemia

reticulocyte high

(COOMBs test)

101
Q

How do you treat spherocytosis?

A

Folic acid

RBC transfusions

splenectomy

(but this doesn’t cure the RBC shape)

102
Q

What are some complications of spherocytosis?

A

gall stones

aplastic crisis

Paige: Heme/Onc Module! (8 decks)

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