Heme Treatment Flashcards
immune thrombocytoenic purpura
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.
heparin induced thrombocytopenia
Discontinue heparin.
Initiate non heparin anticoagulants.
disseminated intravascular coagulation (DIC)
Platelet < 20,000 –> platelet transfusion if not bleeding.
Severe bleeding –> fresh frozen plasma.
Cryoprecipitate.
hemophilia a
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.
hemophilia b
acute bleeding/major surgery –> factor 9 infusions for bleeding episode or prophylaxis.
demopressin not helpful.
type 1 von willebrand disease
mild to mod bleeding, minor procedures –> demopressin
sever, major procedures –> vWF containing product (factor 8 concentrates)
type 2 von willebrand disease
vasopressin but must trial first bc may cause severe thrombocytopenia
type 3 von willebrand disease
only vWF concentrates no demopressin
what triggers the release of vWF proteins from endothelial cells and moves them into the plasma
desmopressin
thrombophilia
anticoagulation at first vte event
factor v leiden mutation
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
protein c or s deficiency
protein c concentrate if acute
indefinite anticoagulation
sickle cell trait
none needed
acute sickle cell crisis treatment
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
sickle cell disease prophylaxis
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.
thalassemia
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
G6PD deficiency
Usually self-limited; avoid offending drugs & food.
Severe anemia - iron and folic acid supplements; blood transfusions if severe.
Neonatal jaundice - phototherapy first line.
Hereditary spherocytosis (HS)
Folic acid
Cholecystectomy
Splenectomy - curative in severe or refractory disease
autoimmune hemolytic anemia Warm
First line: Glucocorticoids
Second line: Splenectomy or Rituximab
Third line: steroid-sparing agents (Azathioprine, etc.)
autoimmune hemolytic anemia Cold
Avoid cold temperature = mainstay
Severe anemia = transfusion, plasmapheresis, IVIG
Rituximab-containing regimens
thrombotic thrombocytopenia purpura (TTP)
Plasmapheresis = initial TOC (Removes antibodies and adds ADAMST13 to serum).
Immunosuppression = glucocorticoids and/or Rituximab if no response to plasmapheresis.
Splenectomy if refractory.
Hemolytic uremic syndrome (HUS)
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.
Paroxysmal Nocturnal Hemoglobinuria
Compliment inhibitors = Eculizumab
Folic acid supplementation
Stem cell transplant
iron deficiency anemia
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.
what do you always give a script for if you are giving a script for iron
stool softner
tips when taking iron
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.
adverse effects of iron
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!
lead poisoning
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
anemia of chronic disease
Treat the underlying disease
Erythropoietin-alpha if renal disease or low erythropoietin levels.
aplastic anemia
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
b12 deficiency
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
folate deficiency
Oral folic acid
If severe, IV folic acid
If patient has B12 deficiency anemia, folate alone won’t help neurological symptoms
hereditary hemochromatosis
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
polycythemia vera (primary erythrocytosis)
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
myelodysplastic syndrome
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
acute myeloid leukemia (aml)
Combination chemotherapy (Cytarabine, Doxorubicin) Hematopoietic stem cell transplant can be curative
leukostasis
leukaphoresis, chemotherapy (hydroxyurea) to remove blasts from plasma
chronic myelogenous leukemia
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.
acute lymphocytic leukemia (all)
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
chronic lymphocytic leukemia (cll)
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.
multiple myeloma
Autologous stem cell transplant most effective
Can be preceded by induction chemo
Chemotherapy usually controls symptoms temporarily
Radiation therapy, bisphosphonates
hodgkin lymphoma
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
non hodgkin lymphoma
Low grade / asymptomatic
no treatment
Localized disease (Stage I)
radiation & chemotherapy
Stem cell transplant if refractory
Intermediate or High Grade (Aggressive) =
Chemotherapy
tumor lysis syndrome
Treatment of electrolyte abnormalities
IV fluids (can add loop diuretic to promote excretion)
Rasburicase or Allopurinol to rapidly reduce uric acid
Hemodialysis if severe
prophylaxis tumor lysis syndrome
Rasburicase (or Allopurinol)
