Heme Treatment

Question 1

immune thrombocytoenic purpura

Answer 1

If no bleeding and platelet > 30,000 –> Observe.

If mild bleeding and platelet < 30,000 –> Steroids, IVIG, Rituximab or splenectomy.

If severe bleeding and platelet < 30,000 –> platelet transfusion and IGIV and high dose steroids.

Question 2

heparin induced thrombocytopenia

Answer 2

Discontinue heparin.

Initiate non heparin anticoagulants.

Question 3

disseminated intravascular coagulation (DIC)

Answer 3

Platelet < 20,000 –> platelet transfusion if not bleeding.
Severe bleeding –> fresh frozen plasma.
Cryoprecipitate.

Question 4

hemophilia a

Answer 4

acute bleeding/major surgery –> infusions of factor 8 BID.
Prophylaxis –> Demopressin to temp increase factor 8. Prior to procedure to prevent bleeding.
Infusions of factor 8 three times a week to prevent joint damage in sever cases.

Question 5

hemophilia b

Answer 5

acute bleeding/major surgery –> factor 9 infusions for bleeding episode or prophylaxis.
demopressin not helpful.

Question 6

type 1 von willebrand disease

Answer 6

mild to mod bleeding, minor procedures –> demopressin

sever, major procedures –> vWF containing product (factor 8 concentrates)

Question 7

type 2 von willebrand disease

Answer 7

vasopressin but must trial first bc may cause severe thrombocytopenia

Question 8

type 3 von willebrand disease

Answer 8

only vWF concentrates no demopressin

Question 9

what triggers the release of vWF proteins from endothelial cells and moves them into the plasma

Answer 9

desmopressin

Question 10

thrombophilia

Answer 10

anticoagulation at first vte event

Question 11

factor v leiden mutation

Answer 11

High risk:
indefinite anticoagulation
lmw heparin during pregnancy to precent miscarriage

moderate risk: (asymptomatic for one thrombotic event that had identifiable, acquired risk factors)
prophylaxis prior to high risk procedures

Question 12

protein c or s deficiency

Answer 12

protein c concentrate if acute

indefinite anticoagulation

Question 13

sickle cell trait

Answer 13

none needed

Question 14

acute sickle cell crisis treatment

Answer 14

Pain management:
IV hydration and O2 (reverses and prevents further sickling)
Narcotic pain medication is appropriate

Folic acid supplementation necessary for RBC production
Transfusion therapy may be needed in some crises

Question 15

sickle cell disease prophylaxis

Answer 15

Hydroxyurea- increases production of hbf (which doesn’t sickle), increases rbc water (reduces sickling), reduces pain, decreases hospitalization and prolonged survival. weeks to months to work so not for acute.
Allogenic stem cell transplant can be potentially curative, but many side effects.

Question 16

thalassemia

Answer 16

Avoid iron supplementation!
Vitamin C and folate supplementation

Transfusions
Regular or episodic transfusions to ensure non-anemic state and prevent some of the disease complications (target Hb = 90-100 g/L)

Iron chelation
Deferoxamine will bind to iron and remove excess iron from the blood (which occurs from chronic transfusions). Avoid iron supplementation!

Endocrine therapy
Administration of deficient hormones and osteoclast inhibitors (bisphosphonates)

Splenectomy
May help to reduce transfusions because no sequestration or destruction of RBCs

Bone Marrow Transplant
May allow body to produce healthy globin and heme

Question 17

G6PD deficiency

Answer 17

Usually self-limited; avoid offending drugs & food.
Severe anemia - iron and folic acid supplements; blood transfusions if severe.
Neonatal jaundice - phototherapy first line.

Question 18

Hereditary spherocytosis (HS)

Answer 18

Folic acid
Cholecystectomy
Splenectomy - curative in severe or refractory disease

Question 19

autoimmune hemolytic anemia Warm

Answer 19

First line: Glucocorticoids
Second line: Splenectomy or Rituximab
Third line: steroid-sparing agents (Azathioprine, etc.)

Question 20

autoimmune hemolytic anemia Cold

Answer 20

Avoid cold temperature = mainstay
Severe anemia = transfusion, plasmapheresis, IVIG
Rituximab-containing regimens

Question 21

thrombotic thrombocytopenia purpura (TTP)

Answer 21

Plasmapheresis = initial TOC (Removes antibodies and adds ADAMST13 to serum).
Immunosuppression = glucocorticoids and/or Rituximab if no response to plasmapheresis.
Splenectomy if refractory.

Question 22

Hemolytic uremic syndrome (HUS)

Answer 22

Supportive therapy = initial management of choice
RBC if severe anemia
Plasmapheresis if severe, neuro complications or renal complications
Antibiotics and anti-motility agents are usually avoided because they may worsen the condition.

Question 23

Paroxysmal Nocturnal Hemoglobinuria

Answer 23

Compliment inhibitors = Eculizumab
Folic acid supplementation
Stem cell transplant

Question 24

iron deficiency anemia

Answer 24

Iron replacement:
Increased reticulocytes (within 5-10 days)
Correction of anemia (6-8 weeks)
Repletion of iron stores (1-3 months)

Preparations:
Iron-enriched food and red meats.
Iron-containing formulas for bottle fed infants.
Oral = Ferrous sulfate
Can give IV if necessary.

Question 25

what do you always give a script for if you are giving a script for iron

Answer 25

stool softner

Question 26

tips when taking iron

Answer 26

for increased absorption: Utilize M-W-F dosing to increase absorption and decrease side effects
Take on an empty stomach OR with water or orange juice (good b/c vitamin C = ascorbic acid -> acid helps break it down for absorption).
Don’t take antacids for 2 hours before taking iron or 4 hours after taking iron.

Question 27

adverse effects of iron

Answer 27

GI (nausea, vomiting, constipation, flatulence, diarrhea, dark stool)

• Educate patients that stool may look dark and it is nothing to worry about
• Always give with stool softeners to help avoid constipation!

Question 28

lead poisoning

Answer 28

Removal of lead source! 
If mild (<44 mcg/dL) -> remove lead source
If moderate (45-69 mcg/dL) -> Oral chelation with Succimer (IV chelation if PO not possible)
If severe (70+ mcg/dL) -> PO and IV chelation

Question 29

anemia of chronic disease

Answer 29

Treat the underlying disease

Erythropoietin-alpha if renal disease or low erythropoietin levels.

Question 30

aplastic anemia

Answer 30

Supportive management is initial treatment of choice (Abx, transfusions, etc.)

For severe aplastic anemia in otherwise healthy patients < 50 y/o:
Stem cell transplant

For patients over 50, or patients under 50 but no matched donor:
Immunosuppressive therapy = Cyclosporine, Prednisone

Question 31

b12 deficiency

Answer 31

Give B12

Routes of administration:
PO, sublingual, nasal and subcutaneous injection

If symptomatic anemia or neuro findings:
Start with SC injections weekly until deficiency is corrected and then once a month.
Patients can be switched to oral therapy after resolution from symptoms
Patients with pernicious anemia need lifelong monthly SC injections
Dietary deficiency - oral B12 replacement

Question 32

folate deficiency

Answer 32

Oral folic acid
If severe, IV folic acid
If patient has B12 deficiency anemia, folate alone won’t help neurological symptoms

Question 33

hereditary hemochromatosis

Answer 33

Phlebotomy is the mainstay of treatment, usually done weekly until:
Ferritin decreased < 50 mcg/dL
Transferrin saturation decrease
Mild anemia

May need maintenance phlebotomy therapy (3-4 times a year for life)

Iron chelation if patients can’t undergo phlebotomy (deferoxamine)

Avoid iron supplementation, vitamin C, alcohol intake

Question 34

polycythemia vera (primary erythrocytosis)

Answer 34

Low risk (< 60 & no history of thrombosis)
Phlebotomy first line until Hct < 45%
Low dose ASA

High risk (60+ and/or thrombosis)
All of the above +
Hydroxyurea (what other disease utilizes this medication?) ____________________
Interferon-alpha = second line
Ruxolitinib (JAK inhibitor) if no response to hydroxyurea

Symptoms
Antihistamines for pruritis

Question 35

myelodysplastic syndrome

Answer 35

Goals are to improve symptoms, improve survival and decrease progression to Acute Myelogenous Leukemia (AML)

Supportive management: may include intermittent blood or platelet transfusions or erythropoietin

Systemic treatment: Pyrimidine analogs

Allogenic hematopoietic cell transplantation only effective cure, but difficult in patients > 50 y/o

Question 36

acute myeloid leukemia (aml)

Answer 36

Combination chemotherapy (Cytarabine, Doxorubicin)
Hematopoietic stem cell transplant can be curative

Question 37

leukostasis

Answer 37

leukaphoresis, chemotherapy (hydroxyurea) to remove blasts from plasma

Question 38

chronic myelogenous leukemia

Answer 38

Tyrosine kinase inhibitors (Imatinib) because they inhibit Philadelphia chromosome tyrosine kinase activity and myeloid leukemic cell proliferation

Allogenic hematopoietic stem cell transplant can be curative.

Question 39

acute lymphocytic leukemia (all)

Answer 39

Combination chemotherapy (produces remission > 85%)
Induction chemo = Anthracyclines, Vincristine and Steroids
Maintenance chemo = 6-MP and Methotrexate.

Relapse = Allogenic hematopoietic stem cell transplant
If CNS disease = intrathecal methotrexate

Question 40

chronic lymphocytic leukemia (cll)

Answer 40

Stage I & II = asymptomatic & indolent
Observation, possibly radiation

Stage III & IV = symptomatic, progressive
Chemotherapy (Fludarabine, Rituximab, Cyclophosphamide)

Acute blast crisis: Tx is similar to AML blast crisis

Allogenic hematopoietic stem cell transplant can be curative.

Question 41

multiple myeloma

Answer 41

Autologous stem cell transplant most effective
Can be preceded by induction chemo
Chemotherapy usually controls symptoms temporarily
Radiation therapy, bisphosphonates

Question 42

hodgkin lymphoma

Answer 42

Stage I or II (early stage)
Combination of chemotherapy and radiation

Stage III to IV (advanced stage)
Combination chemotherapy is mainstay

Refractory = second line high dose chemotherapy & autologous hematopoietic cell transplant are options

Question 43

non hodgkin lymphoma

Answer 43

Low grade / asymptomatic
no treatment

Localized disease (Stage I)
radiation & chemotherapy
Stem cell transplant if refractory

Intermediate or High Grade (Aggressive) =
Chemotherapy

Question 44

tumor lysis syndrome

Answer 44

Treatment of electrolyte abnormalities
IV fluids (can add loop diuretic to promote excretion)
Rasburicase or Allopurinol to rapidly reduce uric acid
Hemodialysis if severe

Question 45

prophylaxis tumor lysis syndrome

Answer 45

Rasburicase (or Allopurinol)