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

Clin Med Diseases (RBC/platelets) Flashcards

(102 cards)

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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
How do you diagnose Hemophilia A?
**PTT** prolonged PT, PFA, fibrinogen level, *platelets NORMAL* **dec. Factor VIII**, C levels vWF is normal
26
What is the tx for hemophilia A?
**infuse** pt with recombinant Factor VIII concentrate\* Desmopressin (DDAVP) to elevate Factor VIII
27
What should patients with Hemophilia A avoid?
aspirin!
28
What is the pathophys of Hemophilia B? | (AKA Christmas disease)
deficiency of Factor IX therefore, also excess bleeding
29
Who gets Hemophilia B and how?
X-linked recessive dz Males **less common** than A
30
What is the clinical presentation of Hemophilia B?
Similar to A! excessive bleeding, bruising, hematomas, hemarthrosis, compartment syndrome
31
How do you diagnose Hemophilia B?
**low Factor IX** **prolonged PT** normal platelet count
32
How do you tx Hemophilia B?
Literally do the same thing as Hemophilia A, except use Factor IX.
33
What is the pathophys of Disseminated intravascular coagulation (DIC)
there is activation of intravascular coagulation there is also increased fibrinolysis therefore, you get both excess clotting AND bleeding
34
What causes DIC?
clotting cascade triggered by: surgery, trauma sepsis, vascular disorders pregnancy, malignancy burns, shock
35
What are the clinical symptoms of DIC?
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)
36
What will lab values show you for DIC? (there is not a huge focus on this anywhere)
prolonged PT prolonged PTT abnormal PFA/low platelets decreased fibrinogen increased fibrin degradation
37
How do you treat DIC?
treat the cause. can give frozen plasma, platelets, or fibrinogen can give heparin to dec coagulation
38
What is the outcome of people with DIC?
most die
39
In patients with clots, will they have high or low D-dimer?
high
40
What is the most common **acquired** coagulopathy?
Vit K deficiency
41
What causes Vit K deficiency?
poor diet, liver failure, malabsorption, malnutrition, some drugs (broad spectrum abx)
42
What are the clinical features of Vit K deficiency?
typically post-op not eating well, receiving broad spectrum abx maybe soft tissue bleeding
43
Lab findings for Vit K deficiency?
PT prolonged PTT prolonged Fibrinogen, platelets normal Levels of Vit K and clotting factors decreased
44
Tx of Vit K deficiency
treat underlying cause. Give Vit K treat hemorrhage with frozen plasma Eat a diet high in leafy veggies
45
What causes iron deficiency anemia?
**Most common** menstruation, pregnancy, **bleeding**
46
What causes folate deficiency anemia?
most likely poor dietary intake (vegan diet)
47
What causes B12 and pernicious anemia?
B12 defiency in diet (**vegans**) Could lack intrinsic factor ("pernicious disorder"), have ileum disorder Some drugs (H2 antagonists)
48
What causes aplastic anemia?
This is a disorder of pluripotent bone marrow stem cells Reduction of RBC, WBC, and platelets Unknown cause
49
What causes hemolytic (G6PD) anemia?
X-linked RBCs break down prematurely, **most common enzyme deficiency (G6PD)**
50
What causes sickle cell anemia?
abnormal Hgb autosomal recessive
51
What do the cells look like in iron anemia?
hypochromic microcytic
52
What do cells look like in folate anemia?
macrocytic megaloblastic
53
What do the cells look like in pernicious anemia?
macrocytic/megalobastic
54
What do the cells look like in aplastic anemia?
hypoproliferative
55
What do the cells look like in hemolytic anemia?
normocytic
56
What do the cells look like in sickle cell anemia?
sideroblastic sickled under stress
57
What are the risks for iron anemia?
pre-menopause pregnancy frequent blood donor diet (milk, Vit C, Zn), family history
58
What are the risks for folate anemia?
alcohol pregnancy some medications (methotrexate)
59
What are the risks for B12 anemia? (pernicious)
being vegan gastric surgery, pancreatic insufficiency, Chron's disease
60
What are the risks for aplastic anemia?
exposure to toxins via certain meds Some blood/autoimmune dz Thymus tumors
61
What are the risks for G6PD deficiency anemia?
being AA or Greek Male stress (inc hemolysis) sulfa drugs, fava beans, nitrofurantoin
62
What are the risks for sickle cell anemia?
being AA (autosomal recessive) (many things can trigger a sickle cell crisis)
63
What is the clinical presentation of someone with iron anemia?
*weakness/fatigue, tachy, exertional dyspnea, pallor* **pica, cheilosis, glossitis** may have **koilonychia** (spoon-shaped nails)
64
What is the clinical presentation of someone with folate anemia?
*tachy, pallor, exertional dyspnea, fatigue* sore tongue (glossitis) **GI symptoms**
65
What is the clinical presentation of someone with pernicious anemia?
*tachy, pallor, exertional dyspnea, fatigue* GI symptoms/malabsorption glossitis **neurological symptoms**
66
What is the clinical presentation of someone with aplastic anemia?
*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
What's the clinical presentation of someone with G6PD anemia?
*tachy, pallor, exertional dyspnea, fatigue* females usually have NO symptoms **jaundice**, dark urine
68
What's the clinical presentation of someone with sickle cell anemia?
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
Diagnostics for iron anemia?
MCV low (microcytic) reticulocytes low (bone marrow problem) Fe, ferritin low TIBC high periph smear: anisocytosis, poikilocytosis
70
Dx for folate anemia?
folate low MCV high low reticulocyte LDH high smear: macro-ocalocytes & hypersegmented polymorphonuclear cells
71
Dx for B12 anemia?
B12 low MCV high low reticulocyte Intrinsic factor Ab
72
Dx for aplastic anemia?
All blood cells low reticulocytes low Fe high TIBC normal Get FULL workup (CBC, liver fn, UA,
73
Dx for g6pd anemia?
periph smear: Heinz bodies, bite cells G6PD low reticulocytes high bilirubin high
74
Dx for sickle cell anemia?
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
Tx for iron anemia?
Ferrous sulfate Find source of bleeding
76
Tx for folate anemia?
Folic acid supplement Avoid alcohol
77
Tx for pernicious anemia?
Vit B12 supplement (IM)
78
Tx for aplastic anemia?
Stem cell replacement (bone marrow/periph blood transplant)
79
Tx for G6PD anemia?
**mostly self-limiting** avoid stress avoid certain medicines and oxidative drugs
80
Tx for sickle cell anemia?
Oxygen, IV fluids, transfusions Children should get LD penicillin daily from birth to 6 years, pneumovax, as well as lots of other screening
81
What is the pathophys of chronic anemia?
Chronic infections/inflammation causes increased hepcidin levels, which **blocks iron absorption in the gut** and **blocks iron release from bone marrow**
82
What causes chronic anemia?
chronic anemia is a sx rather than a disease autoimmune diseases: SLE, RA sickness: cancer, hospitalization
83
What do cells look like in chronic anemia?
microcytic or normocytic normochromic hypoproliferative
84
What are the symptoms of chronic anemia?
*tachy, pallor, exertional dyspnea, fatigue* chronic inflammation
85
How do you diagnose chronic anemia?
Elevated CRP and ESR Normal-high ferritin level
86
Treatment of chronic anemia?
Treat underlying cause Transfusion if symptomatic
87
What will chronic anemic patients not respond to for treatment?
epo or iron supplements
88
What is the pathophysiology of alpha-thalassemia?
deficient synthesis of alpha Hgb chain (low Hgb A) usually leads to RBC hemolysis & increased iron
89
What is the pathophysiology of beta-thalassemia?
deficient synthesis of beta-Hgb (low Hb A2 and low Hb F) usually leads to RBC hemolysis & increased iron
90
What do cells look like in alpha and beta thalassemia?
microcytic hypochromic
91
Who gets alpha and beta thalassemia and how?
Alpha - Southeast Asia and Chinese Beta - African and Mediterranean Hereditary
92
What are clinical features of alpha-thalassemia?
*anywhere from silent to profound defecits* symptoms of iron deficiency in its worst form, can cause stillbirths from hydrops fetalis
93
What is the clinical presentation of B-thalassemia major (Cooley anemia)?
*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
Dx of alpha thalassemia
Hct low Hgb very low **Electrophoresis** shows Hemoglobin H **(confirms)** Periph smear shows target cells, poikilocytes Reticulocytes high Fe, ferritin normal or high
95
Dx of beta thalassemia
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
Tx of thalassemia
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
What is the pathophys of spherocytosis
RBCs are shaped like spears Leads to premature breakdown of RBCs Genetic
98
Who gets spherocytosis?
Northern Europeans
99
What are the symptoms of spherocytosis?
fatigue, irritability, SOB, weakness
100
How do you diagnose spherocytosis?
periph smear - spherocytes bilirubin high CBC for anemia reticulocyte high (COOMBs test)
101
How do you treat spherocytosis?
Folic acid RBC transfusions splenectomy (but this doesn't cure the RBC shape)
102
What are some complications of spherocytosis?
gall stones aplastic crisis

Paige: Heme/Onc Module! (8 decks)

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