Hematology

Front 1

Autoimmune

Hemolytic

Anemia

Back 1

*Systemic disease (SLE, CLL)

*Mycoplasma, drugs

*Fatigue based on degree of anemia

*Elevated indirect bilirubin, no bilirubin in urine< ALT normal

*Coombs positive

* Treat with steroids, cytotoxic agent; refractory cases--> splenectomy, IV immunoglobulins

Front 2

G6PD Deficiency

Back 2

* Acute oxidant stress

* Elevated indirect bilirubin, no bilirubin in urine, ALT normal

* Negative Coombs test

* Bite cells, Heinz bodies

Front 3

ITP

Back 3

* Epistaxis, petechiae, purpura, +/- DI/CNS bleeding

* Low platelet count

* Normal-sized spleen

* Positive antiplatelet antibodies

* Increased megacariocytes on bone marrow
Associated with lymphoma, CLL, HIV, and connective tissue diseases

Front 4

ITP

Treatment

Back 4

1. Steroids (prednisone or dexamethasone for 4-6 wks, followed by gradual taper):

-- Only use in patients with counts <50,000/mm who are at high risk for bleeding

2. IV gammaglobulin (IgG) if acute increase in platalet counts are required with severe bleeding

3. Splenectomy for reccurent disease

4. Subsequent trestments:

-- Danazol or cytotoxic agent

5. Platelet transfusions reserved for acute, unresponsive bleeding

Front 5

Syntetic trombopoetins

for ITP

Back 5

* Romiplostim

* Eltrombopag

Front 6

Von Willebrand

Disease

Back 6

* Platelet-type bleeding

* Normal platelet count

* Prolonged bleeding time, decreased vWF levels, +/- prolonged PTT

* Ristocetin cofactor assay

Treat with DDAVP or Factor VIII replacement

Front 7

DIC

Back 7

* Both platelet-type and clotting-factor type bleeding

* Usually with other major disease

* Elevated PT and PTT

* Low platelets, Positive D-dimer, Elevated fibrin split products, and low fibrinogen levels

Treat with FFP +/- platelets transfusion

Treat primary disease

Front 8

SSD

Treatment Plan

Back 8

1. Agressive pain management with narcotic analgesics

2. Hydration

3. Oxygen

4. Antibiotics (cefotaxime or ceftriaxone) if there is fever or leukocytosis

5. Exchange transfusion if the hypoxia does not correct with supportive measures

6. Hydroxyurea to decrease frequency of crisis in the future

7. Pneumococcal vaccine

8. Chronic folate supplementation

Front 9

Vitamin K

Deficiency

Back 9

* Decreased intake of green, leafy vegetables

OR

* Eradication of flora with antibiotics

* Elevated PT first, then PTT

* Normal platelets

Replace vit K PO, SQ, or IV

Front 10

Factor

Deficiency

Back 10

* Usually just an elevation of either PT or PTT

* Mixing study corrects PT if deficiency;

remains abnormal if inhibitor is present

* Factor XI deficiency - elevated PPT, normal PT, Askenazic Jews

Front 11

Russell viper

venom test

Back 11

* Performed after Mixing study is abnormal

* If abnormal --> LAB fenomen

Front 12

Factor Inhibitor

Back 12

* History of frequent factors VIII or IX replasement

* Elevated PTT +/- PT

* Mixing studies do NOT correct

* Specific antibody testing required

* Overcome with high-volume factor replacement

* Suppress antibody inhibitor with steroids

Front 13

Lupus

Anticoagulant

Anticardiolipin

Antibodies

Back 13

* History of autoimmune disease, HIV infectiion

* No bleeding; thrombophilia

* Recurrent DVT, PE, spontaneous abortion

* False + VDRL and false elevated PTT --> just markers for disease

* Arterial and venus trombosis

* Bleeding time and platelet count normal

* Russell viper venom test prolonged

- No treatment if asymptomatic;

- Otherwise warfarin to target INR of 2-3

Front 14

Major Blood

Group

Incompatibility

Back 14

* Usually a clerical error with immidiate, severe, and life-threatening reaction

* Hypotension, dyspnea, back pain

* Elevated indirect bilirubin; positive Coombs test; fragmented red cells on peripheral smear

* Hemoglobin in urine; free hemoglobin in plasma; low haptoglobin; elevated LDH

-- Stop transfusion; start hydration, mannitol

Front 15

Anaphylaxis

Secondary

to IgA Deficiency

Back 15

* Dyspnea, bronchospasm, hypotension after only a few milliliters of transfusion given

* Reaction to donor IgA

* No hemolysis

-- Give Epi; steroids if severe

-- Use IgA-deficient plasma and washed RBCs

Front 16

Febrile

Nonhemolitic

Transfusion

Reaction

Back 16

* Fever, rigors, and chills with transfusion

* Caused by reacting to antigens or donor WBCs

* No hemolisis, bronchospasm, dyspnea, or urticaria

-- Give acetaminophen acutely

-- Used leukocyte-reduced, filtered blood to prevent

Front 17

Multiple

Myeloma

Back 17

* Bone pain from fractures

* Monoclonal spike in IgG or IgA

* Bence Jones protein in urine

* Anemia and renal falure

* Plasma cells >30% bone merrow biopsy

Front 18

Multiple

Myeloma

tests & treatment

Back 18

-- Best initial test --> Serum protein electrophoresis (SPEP)

-- Bone marrow biopsy confirm the diagnosis

-- Bone scan shows nothing (good for blastic activity, MM is litic )

The Best innitial therapy: Thalidomide and steroids

After --> control disease with allogenic bone marrow transplant

most common cause of death of MM is infection

Front 19

Monoclonal

Gammopathy of

Undetermined

Sygnificance

Back 19

* Monoclonal spike without any of above findings

* No specific therapy

* Monitor for progression to multiple myeloma

Front 20

Coagulopathy

Prolonged PT or PTT

Back 20

Front 21

Think about

bone marrow abnormality

if you see:

Back 21

-- progressive fatigue

-- frequent infections

-- easy bruisability

-- heavy periods

-- B12 or folate deficiency

-- HIV

Front 22

Vit B12

or

Folate Deficiency

Back 22

* For B12 deficiency:

-- low serum levels of vitamin

-- elevated methylamonic acid

* For Folate deficiency:

-- normal methylamonic acid

-- elevated homocysteine level

Front 23

Myelofibrosis

Back 23

* Patients over 50; splenomegalia

* Low, normal or increased WBC count

* Numerous teardrop cells on smear

* Marrow with increased reticulin fibers

* No specific therapy

- Talidomide and lenalidomide increase bone marrow production;

- Ruxolitinib inhibits JAK2 and suppresses myelofibroses

Front 24

Beta

Thalassemia

Minor

Back 24

* Asymptomatic or with mild microcytic anemia

* Profoundly low MCV with NO symptoms

* Increased RBC number, normal RDW, target cells; normal iron studies

* Definitive diagnosis with hemoglobin electrophoresis:

-- increased Hb A2 and Hb F

Front 25

Alpha

Thalassemia

Trait

Back 25

* identical presentation to Beta Thalassemia

* Hb electrophoresis NORMAL

Front 26

Sideroblastic

Anemia

Back 26

* Usually from toxin ingestion, most commonly alcohol, rarely lead

* Microcytic, hypochromic anemia

* Normal ferritin

* Increased serum iron level

* Diagnose with Prussian-blue stain

* Some respond to vit B6 replacement

Front 27

Iron Deficiency

Anemia

Back 27

* Increased blood loss leading to iron store depletio

* Microcytic anemia with decreased RBC number; elevated RDW; low reticulocyte count

* Low ferritin, low iron, increased TIBC

* Treatment is to replace iron

Front 28

Confirmation of

Diagnosis CLL

Back 28

Lymph node biopsy

(not Bone marrow biopsy)

Front 29

Acute Monocytic

Leukemia

Back 29

-- headaches

-- fever

-- weight loss

-- bleeding from the gums or nose

-- occasional skin lesions

* Peripheral smear reveals leukocytosis with high proportion of blast forms

* Alpha-naphthyl esterase positive and is characteristic

Front 30

CML

Back 30

* High WBC count and normal appearing neutrophils

* Philadelphia chromosome and BCR/ABL positive

* Treat with imatinib

* BMT is curative

Front 31

CLL

Back 31

* Eldery

* High WBC is all lymphocytes

* Normal smear exept "smudge cells"

Treat with Fludarabine or Chlorambucil

Front 32

Richter phenomenon

Back 32

Conversion of CLL into high-grade

lymphoma (in 5% of patients)

Front 33

Hairy Cell Leukemia

Back 33

-- Limphocytes have fine, hair-like, irregular projections

-- Bone marrow may become fibrotic

-- Cytochemical feature includes a tartrate-resistant acid phosphatase (TRAP) stain

Treat with Cladribine or Pentostatin

its adverse effects include neurological and kidney damage

Front 34

Epsilon amino caproic acid

Back 34

* Substance that inhibits fibrinolisis

* Used in DIC to increase dissolution of fibrin

Front 35

Aquired storage pool disorder

Back 35

Uremia makes platelets non-functional

Give DDAVP

Front 36

HIT

The most accurate test

Back 36

Platelet factor four antibodies

Front 37

Which mexhanism of

HUS

Back 37

Decrease in ADAMTS 13

Front 38

Best initial therapy

in HUS and TTP

Back 38

* Miled disease -- No tretment

** Severe -- FFP or plasmapharesis

Front 39

Paroxysmal Nocturnal Hemoglobinuria

Diagnostic Tests

Back 39

-- Decay accelerating factor (CD55 and CD59)

-- Ham test

-- Flow cytometry

Front 40

Paroxysmal Nocturnal Hemoglobinuria

Treatment

Back 40

1. Prednison

2. Allogenic bone marrow transplant (cure)

3. Eculizumab - for hemolysis and thrombosis

4. Folic acid and transfusion as needed

Front 41

Tested facts for

Acute Leukemias:

Back 41

Front 42

NHL

Treatment

Back 42

Front 43

HL

Treatment

Back 43

Front 44

Back 44

Front 45

Wandenstrom

Macroglobulinemia

Back 45

* Overproduction of IgM from malignant B cells leading to hyperviscosity

Presents with:

- Lethargy

- Blurry vision and vertigo

- Endorged blood vessels in the eye

- Mucosal bleeding

- Raynaud phenomenon

* Plasmapheresis - best initial therapy

* Rituximab or prednisone - long-term

* Bortezonib or lenalidomide - lower productions of cells