Hemato Week 2 Flashcards by María Siller Rodríguez

General characteristics of vascular and platelet disorders

Bleeding from mucous membranes and into skin
Common petechiae, persistent bleeding from skin cuts
Equal sex

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General characteristics of coagulation disorders

Bleeding into joints and soft tissues
Characteristic deep hematomas, minimal skin bleeding
>80% are male

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Examples of inherited vascular disorders

Hereditary hemorrhagic telangiectasia
Connective tissue disorder → Ehlers-Danlos sx
Giant cavernous hemangioma

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Characteristics of hereditary hemorrhagic telangiectasia

Mutations in endoglin
Dilated microvascular swellings
Recurrent epistaxis and GI hemorrhages

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Characteristics of Ehlers-Danlos syndrome

Collagen abnormalities due to defective platelet adhesion
Hyperextensibility of joints and hyperelastic friable skin

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Examples of acquired vascular disorders

Simple easy bruising
Senile purpura: forearmas and hands
Henoch-Schölein sx: after acute resp tract infection, buttocks and extensor surfaces
Scurvy: vitamin C deficiency
Steroid purpura

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Spontaneous skin purpura and mucosal hemorrhage and prolonged bleeding after trauma

Thrombocytopenia

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Causes of thrombocytopenia

Failure platelet production (most common)
Increased consumption of platelets
Massive transfusion of stored blood to bleeding px

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Most common cause of thrombocytopenia

Chronic idiopathic thrombocytopenia purpura

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Defects in Chronic idiopathic thrombocytopenia purpura (ITP)

Platelet autoantibodies (IgG) result in premature removal of platelets from circulation → antibody directed to GPIIb/IIIa or Ib complex
Lifespan of platelets reduced to hours

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Epidemiology of ITP

Women 15-50 years old

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Labs and tx of ITP

Labs: platelets 10-100 (↓), specific anti-glycoprotein GPIIb/IIIa antibodies
Tx: corticosteroids

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Difference between acute and chronic ITP

Acute: children
Chronic: adults

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Deficiency in thrombotic thrombocytopenic purpura (TTP)

ADAMTS13 metalloprotease

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TTP clinical pentad

Thrombocytopenia
Microangiopathic hemolytic anemia
Neurological abnormalities
Renal failure
Fever

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Dx of TTP

Thrombocytopenia
Schistocytes in blood film
↑ LDH
Absent ADAMTS1
Present anti-ADAMTS13 antibody

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Hemolytic uremic syndrome

TTP with organ damage limited to kidneys
Cause: O157 E coli or Shigella

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Skin and mucosal hemorrhage despite normal platelet count and normal levels of VWF

Disorders of platelet function

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Examples of hereditary platelet function disorders

Thrombasthenia (Glanzmann)
Bernard-Soulier sx
Storage pool disease

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Defect in Glanzmann disease

Failure of primary platelet aggregation
Mutation GPIIb or IIIa

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Defect in Bernard-Soulier sx

Platelets are larger than normal
Mutation GPIb

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Defect in storage pool disease

Grey platelet syndrome
Mutation GPIIb
Platelets larger than normal and absence of alpha granules

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Most common acquired platelet function disorder

Aspirin therapy

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Defect in platelet function with aspirin therapy

Inhibition cyclo-oxygenase with impaired thromboxane A2 synthesis
Effect lasts 10 days

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Drugs besides aspirin that cause platelet function disorders

Pipyridamole: blocks reuptake of adenosine Clopidogrel, prasugrel, ticagrelor: inhibits binding of ADP to its platelet receptor

What causes platelets to be clumped and what to do in that case

Pseudothrombocytopenia due to chelator EDTA Repeat blood count in acid citrate dextrose or heparin

Inheritance of coagulation disorders

Hemophilia A: Sex-linked Hemophilia B: Sex-linked Von Willebrand: Dominant

Deficiency in hemophilia A

Factor VIII

Clinical features of hemophilia A

Post-circumcision hemorrhage Joint and soft tissue bleeds Excessive bruising Prolonged bleeding Painful hemarthrosis and muscle hematomas

Labs in hemophilia A

Abnormal APTT and factor VIII clotting assay Normal PFA-200 and PT

Large encapsulated hematomas with progressive cystic swelling from repeated hemorrhage

Hemophilic pseudotumors

Prophylactic tx for hemophilia A

Emicizumab

Complication in hemophilia A

Development antibodies (inhibitors) to infused factor VIII

Deficiency in hemophilia B

Factor IX deficiency

Defect in Von Willebrand disorder

Reduced or abnormal function of VWF

Binding site of VWF

ADAMTS13

Characteristics of type 2 subtypes of VWF disorder

2A: Decreased platelet function 2B: Increased affinity for GPIb 2M: Decreased binding to GPIb 2N: Normal platelet function

Clinical characteristics of VWF disorder

Mucous membrane bleeding Excessive blood loss from superficial cuts Operative and post-op hemorrhage

Labs in VWF disorder

Abnormal PFA-100 ↓ factor VII, VWF antigen, and collagen binding levels Defective platelet aggregation APTT prolonged Normal platelet count

Tx for VWD disorder

Local measures and antifibrinolytic agent

Causes of vitamin K deficiency

Inadequate diet, malabsorption, warfarin

Coagulation factors affected by warfarin

Factors II, VII, IX, X Proteins C and S

Action of warfarin

Inhibits vitamin K epoxide reductase

Characteristics of hemorrhagic disease of the newborn

Vitamin K dependent factors low at birth PT and APTT abnormal Tx → prophylaxis single IM injection of 1 mg

Inappropriate intravascular deposition of fibrin with consumption of coagulation factors and platelets

Disseminated intravascular coagulation (DIC)

Main clinical feature of DIC

Bleeding

Labs in disseminated intravascular coagulation

↓ platelet count, fibrinogen ↑ levels of fibrin degradation (D-dimers in urine) Prolonged → TT, PT, APTT

Most common acute leukemia in adults (65 yo)

Acute myeloid leukemia (AML)

Classification of AML

* AML with recurrent genetic abnormalities → t(8:21) inv(16) t(15:17) * AML with myelodysplasia-related changes → dysplasia >50% cells in at least 2 lineages * Therapy-related myeloid neoplasm → previously tx with drugs or radiation * Myeloid sarcoma → resembles solid tumor but composed of clustered myeloid blast cells * Myeloid proliferations related to Down sx

Clinical features of AML

Bone marrow failure Frequent infections, anemia, thrombocytopenia Bleeding tendency Gum hypertrophy and infiltration

Labs in AML

Normochromic normocytic anemia with thrombocytopenia White cell count increased, variable number of blast cells, hypercellular bone marrow

Most common genetic defects of AML

t(8:21) and inv(16) → disrupt RUNX1 and CBFB

Genetic defect of acute promyelocytic leukemia

t(15:17): PML fused with RARA

Acute leukemia found in childhood

Acute lymphoblastic leukemia (ALL)

Classification of ALL

B-cell ALL 85% (equal) and T-cell ALL 15% (males)

Pathogenesis of ALL

Started by genetic mutations that occur in utero Mechanism 2nd hit: abnormal immune response to infection

Affected pathway in T-ALL

NOTCH

Clinical features of ALL

Bone marrow failure Organ infiltration → tender bones, lymphadenopathy, moderate splenomegaly, hepatomegaly T-ALL → testicular swelling or signs of mediastinal compression

Labs in ALL

Normochromic normocytic anemia with thrombocytopenia Hypercellular bone marrow (>20% leukemic blasts)

Genetic defects in B-ALL and T-ALL

B-ALL → t(12:21), ETV6-RUNX1 T-ALL → TCR genes show clonal rearrangement

Accumulation in blood of mature lymphocytes that is not cured

Chronic lymphoid leukemia

How is CLL differentiated from monoclonal B cells

>5 x10*9 or tissue involvement

How is the CLL neoplastic cell

Mature cell with weak expression of IgM or IgD

Clinical features of CLL

Males 80% dx from routine blood count Enlargement of cervical, axillary, inguinal lymph nodes

Labs of CLL

Smudge or smear cells CD19+ with expression of only 1 light chain CD5+, CD23+ Reduced serum Igs

Genetic defects in CLL from best to poorest prognosis

del(13q14) trisomy 12 del(11q23) del(17p)

Staging system for CLL

Rai and Binet staging system

Genetic alteration of promyelocytic variant of AML

t(15:17)

Clinical features of promyelocytic AML

DIC and fibrinolysis

Therapeutic treatment of promyelocytic AML

All trans retinoic acid

Morphologic characteristic of AML

Auer rods (needle-like inclusions)

Leukemia with TdT+

ALL

Markers of T-ALL

CD7 and CD8

Markers of AML

CD33 and CD34

Hemato Week 2 Flashcards

(74 cards)