hematology Flashcards
Heme component
FE is part of heme- functions to bind and release oxygen as needed
globin component
5 subunits- in adult hemoglobin- 2 alpha and 2 beta chains- make up the ADULT hemoglobin
cheleation therapy
patients who receive frequent transfusion to remove excess FE
Transferrin and total iron binding capacity (TIBC)
Transferrin binds to free Fe —> transferring trasnports FE throughout the body-
TIBC is indirect way of measuring transferring level:
INCREASED transferring and TIBC : Fe deficiency
Decreased Transferrin and TIBC: Anemia of chronic disease
Ferritin
decreased in FE deficiency
Increased in anemia of chronic disease
Iron is stored in the ferritin protein molecule- IRON IN STORAGE!
Hemolytic anemia (intrinsic)- inherited disorders)
sickle cell anemia, Thalassemia, G6pd deficiency, Hereditary spherocytosis
Extrinsic hemolytic anemia (aquired)
autoimmune hemolytic anemia, DIC, TTP, HUS, hypersplenism
LDH
enzyme found in RBC- too much rbc destruction leads to increase in serum LDH
indirect bili
if too much rbc distruction - causes jaundice
direct bilirubin
dark urine production
sickle cell anemia
hgb S on hemoglobin electrophoresis
Thalassemia
microcytic anemia with normal or increased iron - no tx response to Fe tx.
alpha thalassemia: hgbA, A2 and F- normal hemoglobin ratios
beta thalassemia: hemoglobin electrophoresis shows decreased A, increased A2 and Increased F
G6pd deficiency
episodic- sulfa drugs, fava beans, infections- hemolytic anemia
hereditary spherocytosis
coombs negative and +osmotic fragility test
autoimmune hemolytic anemia
coombs POSITIVE!- microspherocytes
TT{ and HUS: both normal coags
TTP: thrombocytopenia, hemolytic anemia, kidney damage ++++++++ neurological symptoms and fever
HUS: TRIAD!= thromboycotpenia, hemolytic anemia and kidney damage- seen in children- kidney inovelemnt higher than in TTP.
DIC
prolonged PT and PTT
rouleaux formation
stack of coins- multiple myeloma
auto agglutination
clumping of RBC- cold agglutinin autoimmune hemolytic anemia
howell jolly bodies
decreased splenic function !!!
hemolytic cells
bite cells, schistocytes, keratocytes
basophilic stippling
SIDEROBLASTIC ANEMIA, THALASSEMIAS, heavy metal poisioning
target cells
sick cell, thalassemia
hypersegemented neutrophils
B12 and folate deficiencies
Auer rods
Acute myelogenous leukemia
reed sternberg cells
hodkin lymphoma
b12 deficiency
animal food is main source.
b12 binds to IF - absorbed in terminal ileium
-b12 def causs abnormal synthesis of DNA
pernicious anemia- loss of gastric parietal cells- these secrete IF
strict vegans
ETOH
neuro: paresthesia, gait abnormalities, memory loss, dementia, anorexia, diarrhea, glossitis
increase serum omocysteine and methylonic acid
IM b12 repplacement
folate deficiency
needed for DNA syntehsis- no neuro symptoms.
replace folate
iron defi anemia
mc due to bleeding
chronic blood loss- colon cancer or hookworm
pregnancy
crave ice, pica, angular cheilitis, koilonychia (nail spoon)-
decreasedferritin, increased TIBC, decreased iron, decreased transferrin sat
give iron, vitamin C increases FE absoroption
plummer vinson sndreom
dysphagia, esophageal webs, atropic glossitis and fe deficiency
lead poisoining
abd pain with constip, neurologic symptoms, basophilic stippling
cheleation therapy
thalassemia
decreased production of globin chains
after 6 months of age- adult HGB is predominant produced
adult: hgb A: 2 alphas, 2 betas
A2: 2 alphas, 2 DELTAS
fetal: 2 alphas: 2 gamma (tracE)- looks like yY
alpha thalassemia
MC in SE asians
target cells
decreased alpha globin chain production- 4 genes determine it
mild: no treatment
blood transfusion, iron chelating agents
beta thalassemia
decreased production of beta globins chains—>excess alpha chains
minor- 1/2
major: 2/2
minor- no symptoms
cooley’s : major: become symptomatic at 6 monthshepatosplenomagly, severe hemolytic anamie,a jaundice
TARGET CELLS!
increased hgba2 and hgbF
periodic blood transfusions, vitamin C, folate, iron chelating agents.
chronic disease anemia
increased ferritin- iron storage due to acute phase reactant, decreased TIBC, decreased serum Fe
G6pd def
affects mostly males- african american males- heiz bodies-
g6pd usually protects rbc enzyme against oxidative stress- without it- hgb becomes methemoglobin- which does not carry oxygen well. —>these denatured hgb are destructed by splenic macrophages: episodic hemolytic anemia
EPISODIC: splenomegaly- infections, FAV bbeans, SULFA drugs, antimalarials
avoid offending food and drugs
iron/folic acid supp, blood transfusions if needed
sickle cell disease
micro thrombsois- due to rbc sickling because when someone is hypoxic, decreased solubility- so it causes throbosis- sickeld cells then destroyed by the spleen
infections: osteomyelitis: salmonella- aplastic crisis associated with parvovirus!
h shaped ventrebra, splenic sequestration, painful occlusive crisis (acute chest syndrome, back, abd , bone pain)- priapism common
howell jolly- functional asplenia- due to splenic infarction!
TX for sickle cell
IV hydration and OXYGEN!- pain crisis
hydroxyurea: reduces frequency of pain crses
folic acid
immunized for s. neumo, h flu, and n.gonrrhea
STEM CELL TRANSPLANT: cure
hereditary spherocytosis
+osmotic fragility test, hypercrhomic microcytosis, coombs neg, sphre shaped RBC
Tx: folic acid for mild`
splenectomy for severe disease
autoimmune hemolytic anemia
+direct coombs test: distinguishes from hereditary spherocytosis
warm agglutininis: autoimmune- SLE
cold: at colder temp- mycoplasma, EBV
warm: cortico
cold: avoid cold
hemostasis
vascular injury causes platelet aggregatin
activation of that: ADP and thromboxane A2- forms platelet plug
clotting factors- secondary hemostasis
FIBRIN stands- strengthens the platelet plug
Disease: hemophillia, DIC and von willebrand disease
extrinsic path( prolongs PT), intrinsic path (prolongs PTT)
PTT
heparin, DIC, vwD, hemophilia A and B
factors 1,2,5,8,9, 11 and 12
heparin overdose treatment
protamine sulfate
PT
extrinsic pathway: warfarin, vitamin K deficiency, DIC
2, 5,7,10
ttp
thromboycopenia- petechiae, mucocutaenous bleeding- skin, oral,nose bleed,
2. MICROANGIOPATHIC hemolytic anemi
+neurological symptoms and FEVER!
decreased ADAMTS13- vwf cleaving protese: vwf is not cleaved- so it stays there blocking - small vessel thrombosis- RBC become damaged when they circulate in the occluded small vessels- hemolytic anemia
normal coag
splenomeg
TX for TTP
plasmapheriesis- TOC- removes antibodies vs.. ADaMTS13
CORTICOSTEROIDS, cylclophosphamide
HUS
hemolytic uremic syndrome
no fever, or neuro symptoms
preceded by E.coli- shigella or salmonella gastroenteritis
platelet activation by exotoxins: hemolytic anemia, thrombocytopenia
OBSERVATION MOSTLY
TX: plasmapheresis
NO ABX
DIC
bleed and coag
infections!: gram neg sepsis most common reasons, malignancy, or obstetric
thrombosis and widespread hemorrhage
decrease thrombin, decreased fibrinogen, increased PTT/PT/INR- severe thrombocytopenia, increased fibrinolysis, increased D dimer
TReat underlying….FFP if severe bleed- replaces coag factors
ITP
autoimmune thrombocytopenia- mc in children after a viral infection -mucocutaneous bleed- no splenomegally normal COAG TESTS! children: observation, IVIG adults: cortico +IVIG
hemophilia A
only in males- x linked
affects intrinsic of clotting
+HEMARTHROSIS, +excessive hermorrhage in trauma or surgery,
prolonged PTT!!- normal platelet levels
TX: factor 8 infusion
desmopressin - increases factor 8 and vwf
factor 9-deficiency- christmas disases
prolonged PTT- deep tissue bleeding, demopressing IS NOT SUEFUL!!
GIVE FACTOR 9 infusion
Von willebrand disease
ineffective platelet adhesion- - MOST COMMON HEREDITARY bleeding disorder
it is needed for initial platelet adhesions and prevents factor 8 degradation.
mucocutaenous bleed- menorrhagia, epistaxis, easy bruising- PETECHIAe common in VWF
prolonged PTT, bleeding time and ptt prolongation worse with aspirin
mild: no treatment
severe:DEsmopressin
hodgekin’s
bimodal- peaks at 20 and then 50: upper body lymph nodes affected- neck, axilla, sholuder, abdomen
Epstein barr virus
painless lympadenopathy, night sweats weight loss and fever- if advanced
reed-sternberg cells!- mediastinal lymphadenopathy
non-hodgkin’s lymphoma
painless lymphadenopathy
extranodal sites common: GI, skin, CNS MC!!
PERIPHERAL LYMPH NODES MOST COMMON!
multiple myeloma
plasma cells, african american men, bone pain, recurrent infections, elevated calcium, anemia, kidney failure
monoclonal M rotein spike, bence jones proteins, rouleaux formation, punched out lytic lesions
STEM CELL TRANSPLANT!- curative
ALL
C childhood malignancy
FEVER MC symptoms
CNS symptoms too
hepatosplenomegaly, lymphadenopathy!!!
bone marrow: hypercellular with more than 20% blasts!!!, anemia, thrombocytopenia, wbc: 5-100,000
oral chemo: hydroxyurea, stem cell trasnplant
CLL
B cell clonal malignancy
MC leukemia in ADULTS overall
fatigue, lymphadenopathy, hepatosplenomeg
DIFERNTIATED lymphocytes with scattered “SMUDGE cells”, lymphocytosis more than 20k
pancytopenia: thryombocytopenia, anemia
chemo: fludarabine
AML
MC acute form of leukemia in adults
splenomegaly, gingival hyperplasia, LEUKOSTASIS: wBC is more than 100k
AUER RODS!!!! more than 20% blasts!
CML
philadelphia chromosome: splenomegaly!!
more than 100k wbc
more than 30 % blasts
primary erythrocytosis- polycythemia vera
jak 2 mutation
no hypoxia, incresed hemotaocrit- hyperviscosity, pruritis, splenomeg, flushed face
phlebotomy is management of choice
secondary erythrocytosis
hypoxia - pulmonary disease- COPD, high altitude, cyanotic heart disease- reactive!- normal WBC/platelets distinguishes 2nd from 1st.
factor V leidan
MC inheritied cause of hypercoagulability,
- resistant to breakdwon of factor V by cativated protein C- ypecoag- dvt and pe in young patients.
protein c deficiency
vitamin K dependent anticoagulant- stimulates fibrinolysis and clot lysis.
recurrent DVT and pulm emobilism
tx: heparin- anti coag for LIFE
antithrombin III def
venous thrombosis!! recurrent DVT or pulmonary emoboli.
high dose IV heparin!
can lead to heparin resistance because it is activated by heparin
chloromphenicol
chemo tx
SIDE EFFECT: APLASTIC ANEMIA
