Hematology Flashcards
Abciximab, epifibatide, tirofiban
Inhibits GpIIb/IIIa directly
Ristocetin (function)
Activates vWF to bind GpIb.
Failure of aggregantion: vWF D and Bernard- Soulier synd
FFP and prothrombin complex concentrate
Reverse action of warfarin immediately. Can be given with Vit K in cases of severe bleeding
Factor Shortest half life
F (7)
Factor longest Half-life
F (2)
Antithrombin (MOA)
Inhibits thrombin (IIa)
Factors: 7a, 9a,10a,11a,12a
Heparin
Enhances the activity of antithrombin
Principal target of antithrombin
Thrombin (IIa) and factor Xa
tPA
Thrombolytic
Factor V Leiden mutation
Factor V resistant to inhibition by activated protein C
Recurrent pregnancy loss
Iron deficiency
Low iron
High TIBC
Low Ferritin
High RDW
Low RI
Spoon nails (Koilonychia)
Plummer Vinson Synd
Iron deficiency anemia
Esophageal webs
Dysphagia
alpha-thlassemia
a-globin gene deletion on chromosome 16.
Can be cis or trans
A thalasemia
Hemoglobin H disease (- -/ - a) ; excess B globin forms B4
Moderate to severe microcytic hypochromic anemia
A thalasemia
Hemoglobin Barts disease (- -/ - -) ; NO a globin. Excess gamma globin form
Hydrops fetalis; incompatible with life
B THALASEMMIA
Point mutation in splice sites and promoter sequences on chromosome 11. High prevalence in Mediterranean descent.
B thalassemia minor (heterozygote)
B underproduced
Usually asymptomatic
Dx high HbA2 (> 3.5%)
B thalassemia major (homozygote)
B chain absent
Target cells
Anisopoikilocytosis
Marrow expansion (crew cut on skull)
Chipmunk facies
Hepatosplenomegaly
High risk of parvo B19
High HbF and HbA2
Lead poisoning (enzymes inhibition)
Inhibits ferrochelatase and ALA dehydratase
Lead poisoning symptoms
Lead lines on gingivae (burton) and metaphyses
Encephalopathy
Erythrocytes basophilic stippling
Abdominal colic
Sideroblastic anemia
Drops (wrist and foot droop)
Lead poisoning tx
Chelation (succimer)
EDTA
Dimercaprol
Sideroblastic Anemia causes:
X-linked defect ALA synthase
Alcohol
Lead poisoning
Vitamin B6 deficiency
Copper deficiency
Drugs (isoniazid, linezolid)
Sideroblastic anemia findings
High iron
Normal or low TIBC
High ferritin
Ringed sideroblast
Basophilic stippling of RBC
Sideroblastic anemia Tx:
B6 pyridoxine (cofactor of ALA synthase)
Megaloblastic anemia causes:
Vitamin B12 defi
Folate defi
Meds:
- hydroxyurea
- phenytoin
- methotrexate
- sulfa drugs
High homocysteine, NORMAL methylmalonic acid
Folate deficiency
High homocysteine, high methylmalonic acid
Vitamin B12 deficiency
Orotic aciduria cause:
Defect in UMP synthase
Inability to convert OROTIC acid to UMP
Orotic aciduria symptoms
Present in children as:
Failure to thrive
Developmental delay
Megaloblastic anemia refractory to folate and B12
Orotic acid in urine
Hemolysis lead to:
Increased LDH
Low haptoglobin
Reticulocytes
Unconjugated bilirubin
Pigmented gallstones
Urobilinogen in urine
Sickle cell anemia
Point mutation in B-globin gene
Glutamic acid ——> valine
Sickle cell anemia (AR) : electrophoresis
Low HbA
High HbF
High High HbS
Sickle cell anemia Tx:
Hydroxyurea (aumenta la HbF)
Sickle cell anemia complications:
Aplastic crisis (parvo B19)
Autosplenectomy (risk of infection w/ S. Pneumoniae)
Salmonella osteomyelitis
Painful vaso-occlusive crisis
Acute chest Syndrome/ respiratory distress (cause of death)
AIHA (Warm)
IgG
Seen in SLE, CLL
With drugs: b-lactams, a-methyldopa
AIHA (Cold)
IgM
CLL
Mycoplasma
Infectious mononucleosis
Painful blue fingers and toes
Warm AIHA tx
Steroids
Rituximab
Splenectomy (if refractory)
Cold AIHA Tx:
Cold Avoidance
Rituximab
Acute intermittent porphyria (enzyme defect)
Porphobilinogen deaminase deficiency
Acute intermittent porphyria symptoms :
5 P’s
Painful abdomen
Port wine Pee
Polyneuropathy
Psychological disturbances
Precipitated by alcohol, starvation
Acute intermittent porphyria Tx:
Hemin and glucose
Porphyria cutanea tarda: (enzyme)
Uroporphyrinogen decarboxylase deficiency
Porphyria cutanea tarda symptoms:
Cutaneous photosensitivity, hyperpigmentation
Exacerbated by alcohol
Causes: familial, hepatitis C
Chronic Iron poisoning symptoms
Arthropathy
Cirrhosis
Cardiomyopathy
Hypogonadism
Skin pigmentation
PT test
Common (1,2,5,10) and Extrinsic pathway (7)
INR (patient PT/ control PT): use
Most common test to follow pte on Warfarin, which prolongs INR
Normal =1
Prolonged > 1
PTT test
Common an INTRINSIC pathway (all factor EXCEPT 7 y 13)
Hemophilia A
Deficiency factor 8
X- recessive
Intrinsic coagulation defect (high PTT)
Hemophilia B
Deficiency of factor 9
High PTT
X- Recessive
Hemophilia C
Deficiency factor 11
AR
Hemophilia symtoms
Hemarthroses (bleeding into joints)
Easy brusing
Bleeding after trauma (dental procedures)
Vitamin K deficiency
High PT and PTT
Bleeding time normal
Decrease activity of 2 7 9 10 protein C and S
Bernard soulier (AR)
Defect in adhesion
Decrease GpIb
Decrease platelet to vWF adhesion
Labs: abnormal ristocetin test
Large platelets
Glanzmann thrombasthenia (AR)
Defect in aggregation
Low GpIIb/IIIa
Low platelet to platelet aggregation
Labs: no platelet clumping
Immune thrombocytopenia
Low platelet count
Anti- GpIIb/IIIa antibodies
Splenic macrophages phagocytes platelets
Labs: high megakaryocytes on BM
Von Williebrand disease
Intrinsic pathway defect: high PTT
vWF carries Factor 8
AD
High bleeding time
Platelet count: normal
Disseminated intravascular coagulation (DIC)
Widespread clotting factor activation
Low PC
High BT
High PT
High PTT
Labs: high fibrin degradation products (D- dimers)
Low fibrinogen
Low factors V and factor VIII
Heparin overdose
Protamine sulfate
Burkitt
T(8;14) c-MYC
Starry sky
Asso EBV
Jaw lesion
Diffuse large B cell lymphoma
BCL-2 , BCL-6
Mc type in adults
Follicular lymphoma
t(14;18)
BCL2 (inhibits apoptosis)
Indolent
Waxing and waning lymphadenopathy
Mantle cell lymphoma
t(11;14) translocation of cyclin D1
Very aggressive
Marginal zone
t(11;18)
Asso w/ chronic inflammation (sjogren syndrome)
Chronic gastritis
Primary Central nervous system lymphoma
EBV related
HIV/AIDS
CNS mass (ring enhancing lesion)
What cell has the RANKL?
Osteoblast
CO poisoning changes
Increase carboxyhemoglobin concentration
Does NOT affect the PaO2
Left shift of the hb-oxygen dissociation curve.
G6PD deficiency
Decrease NADPH
Reducen glutathione
High RBC susceptible to oxidative stress
Heinz bodies and bite cells
Heparin induced thrombocytopenia type 2 cause:
IgG formation to complexes of heparin and endogenous platelet factor 4.
Major manifestation of HIT type 2
Thrombocytopenia (is due to de destruction of ab-coated platelets by splenic macrophages)
Burkitt lymphoma asso w/ virus ?
Epstein- Barr virus
Burkitt lymphoma
Myc (function?)
Function as a Transcription activator
HbS (valine in place of glutamic) promotes:
Hydrophobic interaction among Hb molecules and results in HbS polymerization and erythrocyte sickling
Döhle bodies
Blue cytoplasmatic inclusions of rough endoplasmic reticulum
Leukocyte alkaline phosphatase is found in:
Maturing neutrophils
Normal or increased in (Leukemoid reaction)
Usually low in CML
Basophilic stippling (made of ? And see in what disorders)
Blue granules (ribosomal precipitates) in the cytoplasm of RBC
See in thalassemia, alcohol use disorder, lead/heavy mental poisoning
Acute hemolytic transfusion (ABO) example of:
Ab-mediated (type II) hypersensitivity
Leads to complement mediated cell lysis
Acute intermittent porphyria tx is Hemin (MOA)
Hemin downregulates hepatic aminolevulinate (ALA) synthase
Heinz bodies made of:
Denatured hemoglobin
Reticulocytes appear as blue- gray on Wright- giemsa staining due to precesence of:
Ribosomal RNA
See in iron deficiency anemia
Tumor lysis syndrome (levels of ions)
Hyperkalemia
Hyperphosphatemia
Hypocalcemia
Tumor lysis syndrome tx:
Hydration
Allopurinol
Rasburicase
