RBC Disorders Flashcards
(33 cards)
1
Q
Thalassemia Clinical Features
A
minor= asymptomatic major= sick
Anemia with normal iron stores
2
Q
Thalassemia Diagnostics
A
- GOLD STANDARD= hemoglobin electrophoresis
- microcytic
- ELEVATED Indirect bilirubin LDH
- DECREASED Hbg (3-6)
- normal/increased iron
- normal TIBC
- Ferritin normal/increase
3
Q
Beta-Thalassemia Clinical Features
A
- sxs begin 4-6months of age when switch from HgbF
- retardation
- abnormal facial structure
- osteopenia
- pathological fractures
- jaundice
- hepatosplenomegaly
4
Q
Thalassemia Treatment
A
- blood transfusions w/iron chelator (deferoxamine)
- bone marrow transplant, splenectomy
- genetic counseling
5
Q
Iron-Deficiency Anemia Characteristics
A
- In adults: SLOW BLEEDING: GI occult bleed secondary to PUD, NSAID, heavy menstrual blood loss
- Low dietary intake of iron in children, impoverished, pregnant
- gastronectomy (decreased absorption)
6
Q
Iron-Deficiency Diagnostics
A
- Decreased H&H
- Smear: hypochromic, microcytic RBC
- DECREASED ferritin (under 20)
- DECREASED iron
- ELEVATED TIBC
7
Q
Iron-Deficiency Treatment
A
- Ferrous Sulfate 325mg tid (titrate up)
- anemia will be fixed in 2 months, but continue therapy for at least 6 months to replenish stores
- iron supplementation during pregnancy and lactation is ESSENTIAL - work-up for occult blood loss
8
Q
Anemia of Chronic Disease Characteristics
A
- chronic inflammation (blocks iron absorption from the gut and the release of iron from the bone marrow)
- RA, SLE
9
Q
Anemia of Chronic Disease Labs
A
- 70% normocytic, 30% microcytic
- elevated CRP
- microcytic
- opposite results of Fe-deficiency (other than Fe):
- DECREASED iron
- INCREASED ferritin
- DECREASED TIBC
10
Q
Anemia of Chronic Disease Treatment
A
- treat underlying cause!
- transfusions while symptomatic
- EPO and iron DO NOT HELP
11
Q
Sideroblastic Anemia Characteristics
A
- reduced hemoglobin synthesis, causing build up of uron in mitochondria= RINGED-CELLS (sideroblasts)
12
Q
Sideroblastic Anemia Causes
A
- lead poisoning
- alcoholism
- decreased copper
13
Q
Sideroblastic Anemia DX
A
- GOLD STANDARD= bone marrow biopsy
- Increased Fe
- ferritin, TIBC wnl
- basophilic stippling of red blood cells
- microcytic
14
Q
Sickle Cell Anemia Patho
A
-autosomal recessive Hgb SS
-sickling only occurs when homozygous
-sickling occurs due to acid, hypoxia, dehydration, temperatures
(AVOID high altitudes, deep sea diving)
15
Q
Sickle Cell Clinical Features
A
- sxs occur at 6months yo (when Hgb F stops)
- vascular occlusions (ischemia causes pain)
- strokes
- chest syndrome
- avascular necrosis
- splenic sequestration
- delayed growth/puberty
16
Q
Sickle Cell Diagnostic Studies
A
- hemoglobin electrophoresis
- during crisis: smear shows sickled cells
- ELEVATED retic count, indirect blilrubin, LDH
- ELEVATED WBC
17
Q
Sickle Cell Treatment
A
- hydroxyurea for SS patients (increases Hgb F)
- Symptomatic during pain crisis: analgesics, hypotonic fluids, rest
- stroke, sequestration, acute chest syndrome may require transfusion/exchange transfusion
18
Q
G6PD Deficiency Patho
A
decreased G6PD means RBC can’t protect against oxidative stress– POPS RBC
19
Q
G6PD Deficiency Oxidative Stresses
A
Cause episodic hemolysis:
- Fava beans
- dapsone, atovoquone (African prophylaxis)
- infection
- quinidine, sulfanomides
20
Q
G6PD Deficiency DX
A
- G6PD 6-8 weeks AFTER event (decrease)
- during episodes:
- ELEVATED retics and indirect bilirubin
- smear: bite cells, heinz bodies (denatured hemoglobin)
21
Q
G6PD Deficiency Treatment
A
- Hemolytic episodes are self-limited and RBC are replaced as soon as offending agent is stopped
- avoid oxidative drugs and fava beans
22
Q
Folic Acid Deficiency Characteristics
A
- usually from poor dietary intake (few WEEKS to deplete stores)
- folic acid is found in green-leafy vegetables
- ALCOHOLICS, anorexia, diet low in fruits/veggies
- pregnancy, chronic hemolytic anemia and exfoliative skin may need supplementation
23
Q
Folic Acid Clinical Features
A
- sore tongue (glossitis)
- GI sxs
- NO neuro sxs
24
Q
Folic Acid DX
A
- macro-ovalocytes and hypersegmented polymorphonuclear cells
- folate level under 150
- NORMAL MMA, elevated homocysteine
25
Folic Acid Treatment
1. Oral replacement (1mg/day)
| 2. avoid alcohol and folic acid metabolism antagonist (trimethoprim, seizure medications)
26
B12 Deficiency Characteristics
- B12 from animal products
1. pernicious anemia most common (lack of intrinsic factor)
2. strict vegan diet
3. Crohns disease
4. Metformin use
27
B12 Clinical Features
1. smooth tongue
2. glossitis
3. cheilosis
4. neuro findings: stocking-glove paresthesias (loss of position, fine touch, vibration), balance problems, dementia
28
B12 DX
1. hypersegmented neutrophils on smear
2. low B12
3. shilling test (rarely done)
4. Anti-intrinsic antibodies
5. ELEVATED MMA and homocysteine
29
B12 Treatment
1. lifelong B12 supplementation IM for pernicious anemia
| 2. neuro signs/sxs are reversible if treated within 6 months
30
Polycythemia vera Characteristics
1. Slowly progressive bone marrow disorder characterized by:
- INCREASED RBC
- JAK2 mutation
2. Increased RBC= hyperviscosity (decreased cerebral flow and hypercoagability)
3. secondary causes: hypoxia, cigarette smoking, living @high altitudes, renal tumors
4. morbidity/mortality from thrombosis
5. may convert to CML/AML
31
Polycythemia vera Clinical Features
1. headache, dizziness, fullness in head/face, weakness, fatigue, tinnitus, blurred vision, burning, pain, redness of extremities
2. pruritis after bathing
3. epitaxis
4. plethora, hypertension, enlarged retinal arteries
5. splenomegaly
32
Polycythemia vera Dx
1. High hemocrit
2. thrombocytosis, leukocytosis
3. hyperuricemia
33
Polycythemia vera Tx
1. GOLD STANDARD: phlebotomy
2. myelosuppressive therapy
3. hydroxyurea
4. low-dose ASA to reduce thrombosis risk
