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Hematology Emergencies. Jack Lazerson, M.D. Professor Emeritus Department of Pediatrics University of Nevada School of Medicine. Clinical emergencies : diagnosis and treatment. The following is an outline, for discussion purposes, that

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Hematology emergencies l.jpg
Hematology Emergencies

Jack Lazerson, M.D.

Professor Emeritus

Department of Pediatrics

University of Nevada School of

Medicine


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Clinical emergencies: diagnosis and treatment

The following is an outline, for discussion purposes, that

will deal with common problems presenting to the physician

as “medical emergency issues”.

A. chief complaint:

1. “bruising for past 24-48 hours”

2. “epistaxsis that won’t stop”

3. “bleeding from ______” e.g. hematuria, melena,

hematochiza

(4. “circumcision bleeding”)


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1. Clinical history including family history

a. Onset and duration of symptom

b. Other symptoms/illnesses and medications

2. Physical examination

a. Type and area of hemorrhagic lesion(s)

b. Other physical findings


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3. Minimum laboratory evaluation

a. Primary mechanism -

PFA 100 or Simplate II Bleeding Time

Platelet Count /MPV/Blood Smear

b. Secondary mechanism -

aPTT/PT/TT

(with 1:1 correction study if test abnormal)


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Mechanism - current concepts (cell-based model)

a. Intiation

b. Amplification (Priming)

c. Propagation

- (“classic” model”)

a. Primary -platelet/vessel wall interation

b. Secondary -fibrin clot formation

c. “Tertiary” - fibrin stabilization

d. “Quatinery” - Fibrinolysis



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Note that these tests are screening tests and

with the history and physical examination can

provide the physician with a presumptive diagnosis

in order to allow for initial treatment, if necessary,

before a definitive diagnosis can be established.


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Common Disorders

1. I.T.P.

2. Thrombocytopenia (non immune)

3. Thrombopathies

4. von Willebrand’s Disease

5. Coagulation Factor Deficiencies

6. Thrombosis


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Common Disorders and Treatment

1. I.T.P.

a. Steroids

b. I.V. Gamma Globulin

c. Anti-D

d. Rituxomab

e. Anit-CD40 ligand


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2. Thrombocytopenia (non-immune)

a. platelets

b. factor VIIa

c. other (E-ACA)

3. Thrombopathies

a. platelets

b. cryoprecipitate

c. DDAVP (Stimate)

d. estrogens


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4. von Willebrand’s Disorder

a. Cryoprecipitate

b. Factor concentrate

(Humate P)

c. DDAVP (Stimate)


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5. Coagulation Factor Deficiencies

a. Plasma

b. Cryoprecipitate

c. Factor concentrates

(VIII, IX, VIIa)


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Thrombosis

1. Risk Factors:

Epidemiological - Aging

Surgery

Oral contraceptives

(Others, including cancer,

pregnancy, etc.

Genetic Predisposition - heterozygote

homozygote


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2. Mechanism of Inhibitors:

Vessel wall

Platelet

Procoagulants

Fibrinolytic

“Other”


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3. Laboratory Assays:

Protein based

DNA based

Thrombomodulin - Heparans

Prostacyclin - Nitrous Oxide

Platelet Glycoproteins

Factor V Leiden (APC Resistance)

Prothrombin 20210A Mutation

Tissue Factor Pathway Inhibitor

Antithrombin 3

Protein C/S

Fibrinogen

Plasminogen

Plasminogen Activator Inhibitor 1

Homocysteine

Antiphospholipid Antibodies (L.A. & A.C.L.A.)

Procoagulants


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4. Treatment:

Anticoagulants

Heparin - unfractionated vs low molecular weight

Coumadin

“Others” - direct antithrombin inhibitors e.g. hiruidin,

agatroban

Antiplatelet agents

Aspirin

Clopidogrel

Monoclonal antibodies

Folic acid


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Advances in Blood Component Therapy

Jack Lazerson, M.D.

Professor Emeritus

Department of Pediatrics

University of Nevada School of Medicine


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Anemia

Chief complaint:

1. “looks pale”

2. “breathing hard”

The minimum evaluation includes the history and physical

examination and baseline laboratory studies.

The CBC must include an absolute reticulocyte count and

peripheral blood smear.

The focused question is whether one needs to transfuse and

therefore what are the indications for transfusion in an

anemic patient?


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Transfusion of red blood cells should be reserved for

the following:

1. Acute blood loss with impending hypotension

2. To prevent or reverse tissue hypoxia (increase organ

perfusion)

(3. Anticipation of progressive anemia)

Note that there is no arbitrary level of hemoglobin or

hematocrit as an indication for transfusion.

Packed red blood cells are the product of choice with the

notation that CMV negative, washed, leukoreduced and

irradiated cells may be indicated.


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Red Blood Cell Transfusions

1. Indications

Acute Blood Loss

Chronic Transfusion Theory

2. Packed Red Blood Cells

Leukoreduction of RBCs

Irradiation of RBCs


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Erythropoietin

Stimulates

a. proliferation

b. differentiation

Requires adequate iron intake and stores


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Chief complaint:

1. “bruising for past 24-48 hours”

2. “epistaxsis that won’t stop”

3. “bleeding from ______” e.g. hematuria,

melena, hematochiza


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3. Minimum laboratory evaluation

a. Primary mechanism -

PFA 100 or Simplate II Bleeding Time

Platelet Count /MPV/Blood Smear

b. Secondary mechanism -

aPTT/PT/TT

(with 1:1 correction study if test abnormal)


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Platelet Transfusions

1. Indications

2. Dosage

1 unit/10 kgm


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Thrombocytopenia (non-immune)

a. platelets

b. factor VIIa

c. other (E-ACA)

Thrombopathies

a. platelets

b. cryoprecipitate

c. DDAVP (Stimate)

d. estrogens


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Blood Coagulation Factors

1. Plasma

2. Factor VIII

3. Factor IX

4. vW Factor

5. Factor VIIa


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von Willebrand’s Disorder

a. Cryoprecipitate

b. Factor concentrate

(Humate P)

c. DDAVP (Stimate)


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VIDEO

Part I


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Apheresis

1. Manual

2. Automated

a. Continuous

b. Discontinuous


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