heme Flashcards
what is the most common anemia in the world? what causes it?
what about in america?
iron deficient anemia!!
MOSTLY FROM PARASITES!! GROSS
In america: menses
Iron Deficient Anemia
what size and color? what two unique characteristics about this cell type hint: shape? what are 3 main causes? what should you always suspect? what are 5 unique presentations you might see on PE? what is the treatment? for how long?
hypochromic, microcytic anemia (since no iron to give shape or color)
anisocytosis (unequal size) and piokilocytosis (tear drop shapped)
common causes: blood loss (menses, occult from colon, esophagus, stomach), pregnancy, vegan diet
**always suspect malignancy**
pica (eating dirt/paint), cheilosis, koilonchia “spoon nails”, glottitis “smooth tongue”, esophageal webs, pallor, tachycardia
treatment: Ferrous sulfate 325 mg 3x a day, vitamin C to make absorb better and uptitrate, OR GLUCONATE which is IM or IV
TREAT FOR 6 MONTHS!!
what can cause blood loss in iron deficient anemia? 4 things
GI blood loss from NSAIDS, PUD, cancer
blood donation
trauma
menses
what would you see on the labs for iron deficient anemia?
5 things!
- Low Iron <50
- High TIBC (since none to bind, very avaliable)
- low ferritin (since none to store)
- low reticulocyte
- hypochromic, microcytic cells
what is the number one cause of iron deficient anemia? what are 3 other causes?
- blood loss! need to find the cause!!
2. malignancy! need to think about this
- dietary, vegan!! less common
- poor iron absorption/ trauma
what is this and what condition do you commonly see this with?
hand and foot syndrome seen with sickle cell
commonly the first presentation
soft tissue swelling with new bone formation and moth eaten lytic process at proximal aspect of fourth phalanx
no leukocytosis or erythema with the swelling
sickle cell anemia
what is the inhertiance pattern? what is the difference between heterozygous/homozygous? when do the problems first occur? where is the mutation? what are 8 things that can prompt sickeling? what are 7 presentations you can see with this disorder?
autosomal recessive
heterozygous 1 Hb S gene: 40%
homozygous 2 Hb SS gene: 80-95%
problems start about 6 months after birth during transition from Hb-F to Hb
mutation in B chain, cause it to sickle under/from:
dehydration, hypoxia, acidosis, infection, temp changes, exertion, alcohol, medical procedures
causes acute painful syndrome, acute chest syndrome, splenic sequestration, aplastic crisis, hemolytic crisis, hand foot disease, “silent” cerebral infarction (35%, subtle but permanent)
what is the goal for the Hb and HbSS for patients with sickle cell?
Hb>10%
HbSS <30%
helps to determine when transfusion or intervention are required
explain the pathphys of sickle cell
increased RBC destruction
inability to maintain hemoglobin
sickling of cells=increased blood viscosity and ostruction
MORE FRAGIL=hemolysis!
what is the life expectancy for a pt with sickle cell?
40-50 years
die young from infections
Explain actute painful crisis and acute chest syndrome seen in sickle cell pts
acute painful crisis:
excrutiating, can occur anywhere
acute causes vasco occlusion and ishchemia
acute chest syndrome
25% of deaths!
respiratory distress
explain aplastic crisis seen in sickle cell and the five things that can cause it?
stop of RBC production, and since their RBC live so much short ~20 days, they get EXTREME drop in hemoglobin causing aplastic crisis
parvarovirus B19, infection, bone marrow toxins HPV, folic acid deficiency
explain the hemolytic crisis seen in sickle cell and what patients with another disease is this commongly seen with? what two things can prompt this?
higher rate of hemolysis than normal
frequently in patient with G6PD deficient
actute bacterial infection/oxidatative drugs
what do sickle cell patients need to avoid?
altitiudes over 7,000 feet and deep sea diving!
induces sickling
what do you see for lab results for a patient with sickle cell? 6 things!!
- howell jolly bodies
2. Hgb S >50%
3. hb 6-8
4. RBC last 10-20 days
- high reticulocytes
- high ferritin/serum bilirubin
what treatment options are avaliable for someone with sickle cell?
- hydration
- pain meds!!
- transfusion
- folate supplementation
- iron chelation if Fe overload
- preventative vaccines for S. pneumonia, H. influenzae
-prophylatic penicillin from birth to 6 years
hydroxyurea
explain hydroxyurea for sickle cell patients 3 things
- Decrease DNA synthesis
- Inhibit sickling
- Increase Hb F inhibits Hb sickling
**prevents complications and increases life span**
what are sickle cell patients are increased risk for?
(6 things)
- infection with encapsulated organisms
- aseptic necrosis
- CVA
- chronic leg ulcers
- splenic infarc THESE PATIENTS ARE ASPLENIC (DON’T HAVE A SPLEEN THAT WORKS WELL SO NEED TO MAKE SURE THEY ARE VACCINATED ESP AGAINST STREP PNEUMONIA
- retinopathy
explain HbSC and HbSS in sickle cell
Hb SC is the trait for sickle cell, heterozygous and range of symptoms vary
Hb SS the disease for sickle cell, homozygous, severe disease
sideroblastic anemia (lead poisoning)
what are the size of the cells? reticulocyte count? platelets? what are three unique lab results you will see with lab testing? what will the patient present with for symptoms? what do you do for treatment depending on the levels of lead in the blood? what does the location of the lead tell you about how long it has been in the body? what does the bone marrow produce?
microcytic, decreased reticulocytes, low platelets
basophilic stippling, elevated lead, erythrocyte protoporphyrin, bone produces ringed sideroblasts instead of health RBC
PE: lead on the gum lines, vomiting, abdominal pain
high serum levels: acute attack
if in the bone: hard to tell how long its been there
BLL>20, medical and environmental intervention
BLL>45, chelation
what do you need to caution for in children with lead poisoning aka sideroblast anemia? what happens in this?
reduced hemoglobin synthesis cause iron accumulation esp in mitochondria
watch for neurological symptoms in children
beta thalaseemia
what chain is effected in this? what parts of the world is this common in? what unique cell do you see in the lab results? what are the two main classifications of B thalaseemia? when is the more severe on diagnosed? what are four clincial presentations of this? what are 3 treatment options? what do you want to keep [hb] at? what test do you use to tell the difference between Fe and thallessemia?
deficient synthesis of B-globin chain of hemeoglobin
(results in increase A) African/mediteranneans
HEINZ BODY CELLS!! “Target cells” “HAIR ON END APPEARANCE ON XRAY”, FRONTAL BOSSING
minor: heterozygous, sufficient Hb sythesis
major: homozygous! SEVERE transfusion dependent anemia, diagnosed 1st year of life when hb F turns to Hb A (adult) “cooleys anemia”
growth retardation, hepatosplenomegaly, abnormal facial formation, fractures/osteopenia, delayed or absent puberty, hypogonadism
Tx: regular blood transfusions to keep hemoglobin at 12 mg/dl, avoid Fe supplements, bone marrow transplant/splenectomy
MENTZER INDEX
xalpha thalessemia
what are the deficient in? what type of cells can be present? what nationality of people are most common? explain the four stages? size and color? what lab results remain normal? what do you treat with? what should you avoid?
deficient a-globin chain, 4 stages target cells Heinz bodies!!
ASIANS
usually diagnosed if iron supplemets for suspected iron deficient don’t work
- silent carrier, 1 gene deleted
- trait, 2 gene deleted leading to mild hemolytic anemia
- Hb “H”, 3 genes deleted, hemolytic anemia without transfusion need
- lethal at birth, hydrops (seen in pic)
microcytic hypchromic but not very anemia, normal iron, TIBC, ferritin
Tx: folic acid supplement, avoid iron, if that doesn’t work then transfusion but not dependent like B thalassemia
which one is more severe B thalmassemia or A thalmessmia?
B thalmassemia because the accumulation of A chains is more toxic
how are the thalassemias named?
what are the general treatments listed by professor? (4 treatments)
FOR WHAT THEY ARE DEFICIENT IN!!!
1. blood transfusion with chelation
2. hydroxyurea (increases HbF)
3. bone marrow transplant
4. splenectomy
von willebrand thrombocyte
what type of genetic inheritance does this show? what happens in this? explain the 3 types? where do you see the bleeding? what are 4 lab findings (3 abnormal)? what pathway does this effect? what are the 2 treatment options?
deficient/defect in vWF, autosomal dominant
deficiency vWF: doesn’t stabalize factor VIII or allow platelets to stick to the vessel wall for clotting
prolongs bleeding time! RISTOCETIN ACTIVITY IS THE GOLD STANDARD TO TEST (ABX that tests coagulation in vitro)
type 1: most common, mild bleeding 75-80%
type 2: vWF abnormal
type 3: rare, most severe, low vWF and factor VIII
bleeding: nasal, sinus, vagina, GI, menses
LABS: LOW vWF, PT NORMAL, PFA (PLATELET FUNCTION ABNORMAL), PTT ABNORMAL SINCE EFFECTS THE INTRINSIC PATHWAY WITH FACTOR VIII
TX:
- Cryoprecipitate (plasma with vWF and VIII)-surgery/complications
- vaspressin DDAVP/vasopressin (stimulates release of vWF from endothelia cells)
hemophilia A “classic”
what genetic heredity type is this? what happens in this? what do you need to differentiat it from and how do you do that? what are four clincial presentations and what is the key one? what 3 tests are improtant? what are the two treatment options?
deficiency of Factor VIII which is needed for clotting, x-linked recessive males
excessively long clotting time, most severe bleeding disorder
hemarthrosis (KEY!!), bleeding after circumcision, intracranial bleeding, compartment syndrome (increased pressure in arm/leg/confined space) epitaxis, bleeding into small tissues,
**include neuro bleeding and hemarthrosis/compartment syndrome**serious bleeding
factor VIII low, PTT prolonged, vWF normal(differentiates with von wilebrand), normal PT, PFA, fibrinogen, platelet count!!
TX: fresh prozen plasma, recombinant factors, prophylaxis with recombinant factor VIII, desmopression (increases VIII)
hemophilia B
“xmas disease”
what is the deficency here? what is the genetic hereditary? males or females? where are 3 common places to have bleeding? what are two important lab results? what are two treatment options?
deficiency of factor IX, x-linked recessive
MALES
hematomas, hemarthrosis, compartment syndrome but different factor than A!!
Factor IX low, PTT increased (since intrinsic pathway deficiency), platelet count normal
Tx: fresh frozen plasma, recombinant factors, prophylaxis with Factor IX, on demand factor replacement
Acute lymphacitic leukemia (ALL)
what is this the most common of? what subsets of cells does it involve? what will you see on the smear? what condition is it associated with? what is one caution you NEED to address when treating this? what is used for diagnosis and what is the treatment?
1 cause of cancer in children!!
very young: 3-7 yrs
associated with down syndrome
T or B lymphblasts subsets
**hides in CNS so must treat with prophylaxis!
bone pain since ramped up
DX: smear, BM biopsy
TX: 3 phases of chemo!! prophylaxis chemo Ara-c for cancer hiding in CNS, LUMBAR PUNCTURE FOR CNS INVOLVEMENT!!!
what is a patient with Acute lymphocytic leukemia at risk for developing later on in life?
Acute myeloid leukemia beause they were exposed to chemo so their myeloid line can be effected later on
what is the most common pediatric cancer?
acute lymphocytic leukemia!!
explain the two T and B cell subsets of acute lymphocytic leukemia!!
what are the chromosomes that are effected in B cell ALL? what are the symptoms associated with T cell ALL?
This was from khan academy and meded
Acute myelogenous leukemia (AML)
who do you see this in? what are two important things you will see on the SMEAR? what percent achieve remission? what are the three treatment options? what exposure do you usually have to get this?
adults, acute picture
>20% blasts, AUER ROD CELLS, bone pain since ramping up cells
WBC>100,00
70% achieve remission
EXPOSURE: BENZO, CHEMO, RADIATION
TX: combination chemo, bone marrow transplant vit A
