Hematology Medical Management Flashcards
(80 cards)
1
Q
What is polycythemia?
A
the body makes too many RBCs making the blood thicker and causing clots
2
Q
what is Secondary Polycythemia?
A
response to chronic hypoxemia which triggers increased production of erythropoietin by the kidneys
3
Q
what are some common causes of polycythemia?
A
- obstructive sleep apnea
- obesity hypoventilation syndrome
- COPD
- testosterone replacement therapy
- heavy cigarette smoking
4
Q
In the neonatal ICU, polycythemia is common and is treated with
A
partial exchange transfusion if the baby is symptomatic or has a hematocrit >75%
5
Q
What is the WHO criteria for anemia?
A
- <13 g/dL in men
- <12 g/dL in woman
- <10.6 g/dL in children 2 yrs or younger
6
Q
what are the signs and symptoms of anemia?
A
- Usually slow onset and few symptoms
- fatigue, palpitations and shortness of breathe common
- glossitis and angular cheilitis
7
Q
what are the MCVs for microcytic, normocytic and macrocytic anemias?
A
Micro: MCV < 80
Normo: MCV 80-100
Macro: MCV > 100
8
Q
what are the microcytic, normocytic and macrocytic anemias
A
- Micro: iron deficiency, thalassemias, lead poisoning
- Normo: hemolytic, renal failure, blood loss, chronic diseases
- Macro: B12 and folate deficiencies, hypothyroidism
9
Q
what are the laboratory findings for Iron Deficiency Anemia?
A
- low serum ferritin
- low serum iron
- High transferrin and Hgb total iron binding capacity
10
Q
what are the treatments for Iron Deficiency Anemias?
A
- stop the source of bleeding
- In children: check lead
- treat with iron supplementation (oral)
11
Q
what is an abnormal lead level and what are the therapies for high lead levels?
A
abnormal lead level >3
- >10 treatment is abatement and iron supplementation
- >45 treatment is chelation therapy using Succimer (watch for increase in ALT/AST), Dimercaprol (avoid in G6P def.), CaNa2-EDTA (watch for renal issues)
12
Q
what would you check for in a person with B12 Deficiency Anemia?
A
check folate, B12, and metabolic intermediate (methylmalonate and homocysteine) levels
* schilling test: radioactive B12 absorption test (not used really)
13
Q
what is the treatment for B12 Deficiency Anemia?
A
supplement with vitamin B12 (oral or IM cyanocobalamin; dietary)
14
Q
what is Pernicious Anemia?
A
a type of vitamin B12 deficiency anemia caused by autoimmune destruction of parietal cells in the stomach which make intrinsic factor for B12 absorption
15
Q
what does a G6P deficiency lead to a build up of?
A
oxidants and precipitation leads to Heinz bodies
16
Q
what can trigger an increase in hemolysis in G6P deficient anemic patients?
A
- ASA (Aspirin)
- Phenacetin (old painkiller, rarely used today)
- Dapsone (antibiotic, used for some infections)
- Ascorbic acid (high doses of Vitamin C)
- Vitamin K (in high doses)
- Fava beans
17
Q
what are the preventative treatments for Sickle Cell Anemia?
A
- Hydroxyurea: increases total and fetal hemoglobin, also used to halt RBC, leukemia cells and platelets
- Long-term, periodic blood transfusions
- Immunizations (Streptococcus pneumoniae, Haemophilus influenzae, hepatitis B, and influenza)
- L-glutamine oral powder (Endaril) which reduces oxidative stress
18
Q
what are the treatments for Acute Crisis Sickle Cell Anemia?
A
- rapid pain management
- IV hydration
- antibiotics
19
Q
How would you diagnose Thalassemia?
A
CBC, MCV (low 60s), hemoglobin electrophoresis
20
Q
What are the causes of Aplastic Anemia?
A
often unknown but may be due to,
- drugs (anticonvulsants, antibacterials etc)
- viruses (EBV, B19V)
- genetics (Fanconi’s anemia)
- exposures to benzene, radiation etc
21
Q
what are the symptoms of Aplastic Anemia?
A
symtpoms from anemia and thrombocytopenia PLUS
- petechiae, ecchymoses, gingival bleeding
22
Q
what is the treatment for Aplastic Anemia?
A
- Stop inciting cause, if possible
- May resolve after hepatitis B infection, Parvovirus B19 (red cell only)
- Depends on how severe
- Mild -> often no care or supportive care only
- Severe -> antibiotics, transfusions, growth factors, stem cell transplant
23
Q
What can renal failure anemia lead to?
A
- erythropoietin deficiency
- normochromic, normocytic
24
Q
How would you diagnose Acute myelogenous leukemia (AML)?
A
- mainly ADULTS
- Peripheral blood and bone marrow stained with Wright-Giemsa
- At least 20% myeloblasts in bone marrow or peripheral blood
- Stain positive for myeloperoxidase
- Immunotype-positive for markers
25
How would you diagnose Acute Lymphoid Leukemia (ALL)?
- mainly in CHILDREN
- Massive replacement of bone marrow space with leukemic blast cells
- Anemia, thrombocytopenia
- 3 distinct subtypes based on type and size of neoplastic lymphocytes: L1, L2, L3
26
what is the medical management plan for Acute Leukemias?
- cure is related to tumor burden and the rapid elimination of malignant WBC
- 3 phases of chemotherapy; induction, consolidation/intensification, complete remission/maintenance therapy
27
what is the criteria for complete remission in Acute Luekemias?
- Platelet count > 100,000/μL
- Neutrophil count > 1000/μL
- Bone marrow specimen with < 5% blasts
28
what are sanctuary sites?
when leukemia cells migrate to areas in the body where chemotherapy agents cannot reach them
*** in ALL the most important sanctuary site is CNS
29
what is the stage of Chronic Myelogenous Leukemia (CML) that has the best prognosis and what age group is mainly effected?
- chronic stage with <10% blast cells
- median age of diagnosis is 67 years old
30
when are most patients diagnosed with CML?
during the chronic stage
31
what are the medical treatments of CML in the chronic stage?
- Interferon-α or imatinib mesylate (Gleevec), (inhibitor of tyrosine kinase) can obtain complete remissiion in 70% of patients in 5 years
- Stem cell transplant can obtain remission in >70% at 10 years if caught before accelerated or blastic phase
32
what is the most common type of leukemia in adults?
Chronic Lymphocytic Leukemia (CLL)
32
How is CLL classified?
BINET System;
- Stage A: two or fewer lymph node groups, no anemia or thrombocytopenia
- Stage B: three or more lymph node groups, no anemia or thrombocytopenia
- Stage C: anemia and thrombocytopenia, any number of lymph node groups
33
what is the medical treatment for CLL?
Not curable; treatment has little effect on survival times
34
what are the mean survival times for CLL?
- Stage A: >10 years
- Stage B: 5 years
- Stage C: 2 years
35
where do initial signs of lymphomas usually occur?
in the mouth
36
how is Hodgkins Lymphoma diagnosed?
- nodal biopsy or bone marrow aspirate
- large, multi-nucleated Reed Sternberg cells present
37
what is the cure rate for Hodgkins Lymphoma?
90%
38
What is the treatment plan for Hodgkins Lymphoma?
- chemo
- radiation therapy
- stemcell transplant if relapse occurs after chemo and radiation
39
what is the chemotherapy regimen given for Hodgkins lymphoma if radiation therapy was given alone at first?
ABVD regimen
- Adriamycin (doxorubicin)
- Bleomycin
- Vinblastine
- Dacarbazine
40
what is the main cause of most Non-Hodgkins Lymphoma cases?
More than 80% are B cell origin
- 60% of cases involve diffuse large B cell and follicular lymphomas
41
How is Non-Hodgkins lymphoma diagnosed?
- Excisional biopsy of involved lymph node
- Staging of disease requires complete blood cell count, chemistry, chest X-rays, CT scans, and bone marrow biopsy
42
What is the treatment plan for Follicular Lymphomas?
- cancer cells are easily killed by radiation therapy
- watch and wait
- most patients have extensive disease at diagnosis (8-10 year survival rate)
43
What is the treatment plan for Diffuse Large Cell Lymphoma?
- 30% have stage I or minimal stage II
- chemo then radiation
44
what lymphomas are Waldeyer's rings present in?
Commonly involved in Non-Hodgkins
- rarely involved in Hodgkins
45
where are most tumors located in Multiple Myelomas?
skeletal system
46
what does Multiple Myeloma look like on a radiograph?
Multiple “punched- out” lesions or mottled areas
47
what is the most common symptom of MM?
persistent bone pain and hypercalcemia leading to headaches and peripheral neuropathy
48
what are common laboratory findings for MM's?
- osteolytic bone lesions
- abnormal immunoglobulin light chains in the URINE (bence-jones proteins)
49
How is MM diagnosed?
protein electrophoresis of serum or urine to detect myeloma/monoclonal protein band (IgG mainly)
50
what is the therapy used to treat MM?
corticosteroids (Prednisone, Dexamethasone) but can lead to increased blood sugar and appetite, problems sleeping and infections
51
when is a bone marrow transplant used for leukemia treatment?
depends on the disease state and used in combo with cyclophosphamide, total body irradiation, busulfan
52
what are the preoperative preparations for a bone marrow transplant?
- allogeneic marrow graft: best if sibling (identical twin is best case)
- autologous marrow graft: using patients own marrow
53
what is Graft Versus Host Disease (GVHD)?
- lethal complication of allogeneic transplants can be either acute (within first 3 months) or chronic (after 100 days)
54
what are the main clinical presentations of bleeding disorders?
petechiae and ecchymoses
55
what are the screening tests for bleeding disorders?
- aPTT (intrinsic)
- PT (extrinsic)
- Platelet count
- TT
56
what are the vascular defects that lead to bleeding disorders?
scurvy
small vessel vasculitis
57
what are the platelet function defects that lead to bleeding disorders?
- aspirin inhibits thromboxane synthesis
- other drugs hurt platelets (NSAIDs, beta-lactams etc)
- uremia causes build up of waste and platelets do not work correctly
58
what is disseminated intravascular coagulation (DIC)?
clotting system is activated in all or a major part of the vascular system leading to a decrease in clotting factors which in turn causes uncontrolled bleeding
59
what is the difference between acute and chronic DIC?
- Acute: Severe bleeding from small wounds, purpura, and spontaneous bleeding from nose, gums etc
- Chronic: thrombosis is more common than bleeding
60
How is DIC diagnosed?
- a prolonged PT, aPTT and TT
- thrombocytopenia
* chronic DIC difficult to diagnose
61
what is the treatment for DIC?
- reverse the cause, control of the major symptom (bleeding or thrombosis), and a prophylactic regimen to prevent recurrence in cases of chronic DIC
- Replace consumed coagulation factors; restore fibrinogen levels
- Cryoprecipitate if bleeding is the major problem
- Fresh frozen plasma
- IV heparin (longterm infusion for chronic)
62
what are the main causes for venous and arterial thrombosis?
- Cancer (malignancy)
- Hormonal therapy
- DIC (disseminated intravascular coagulation)
63
When do you evaluate for inherited thrombotic disorders?
- Younger than 45 years of age and have recurrent thrombosis
- Single thrombotic event and a family history of thrombosis
64
what are the treatments for arterial thrombosis and venous thrombosis?
- Arterial: antiplatelet drugs like Aspirin and Clopidogrel
- Venous: Treat with drugs that prevent thrombin formation (important clotting enzyme) and break down fibrin clots if needed
65
what are the antidotes for Heparin and Warfarin overdose?
- Heparin overdose →give protamine sulfate
- Warfarin (Coumadin) overdose →give vitamin K
66
what are the anticoagulant Direct Factor Xa inhibitors?
*(xaban)
- Rivaroxaban (Xarelto)
- Apixaban (Eliquis)
- Edoxaban (Lixiana)
67
what are the anticoagulant direct thrombin inhibitors?
** (din, tr)
Lepirudin
Desirudin
Hirudins
Argatroban
Dabigatran (Pradaxa)
68
what are the INR Goals for Warfarin (Coumadin Monitoring)?
- low intensity (2-3) for Venous thrombosis, Pulmonary embolism (PE), Atrial fibrillation
- high intensity (2.5-3.5) for Mechanical heart valves, Antiphospholipid antibody syndrome
69
what are the anti-platelet drugs?
- Aspirin
- Aspirin plus dipyridamole (Aggrenox)
- NSAIDs
ADP inhibitors
- Clopidogrel (Plavix)
- Ticlopidine (Ticlid)
GP IIb/IIIa inhibitors
- Tirofiban (Aggrastat)
- Abciximab
- Eptifibatide
70
how is Hereditary Hemorrhagic Telangiectasia (HHT) characterized?
Osler-Weber-Rendu syndrome multiple telangiectatic lesions involving the skin, mucous membranes, and viscera especially on and under the tongue, toes and fingers
71
what are the treatments for HHT?
- Embolization of arteriovenous communications
- Hormonal therapy (estrogen or estrogens plus progesterone) for epistaxis
- Pulmonary resection or embolization for pulmonary arteriovenous malformations
- Hormonal therapy and laser coagulation for GI lesions
72
what is the most common form of Von Willebrand disease and what does it mostly present as?
- type I (70-80%) and mainly presents with signs and symptoms of hemophilia A
73
what are the laboratory findings for Von Willebrand disease?
- Prolonged aPTT
- Normal platelet count, prothrombin time (PT), thrombin time (TT)
- Additional tests to confirm diagnosis and type; Ristocetin cofactor activity, ristocetin-induced platelet aggregation
74
what is the treatment for Von Willebrand Disease?
- Cryoprecipitate
- Factor VIII concentrates with vWF
- Desmopressin: vasopressin analogue that increases factor VIII activity given IV or nasally 1 hour before surgery
75
what are the Hereditary Platelet Function Disorders?
- Bernard-Soulier Syndrome: Platelets are large and defective and cannot bind to von Willebrand factor (vWF) → poor platelet adhesion
- Glanzmann’s Thrombasthenia: Rare autosomal recessive disorder where platelets cannot bind fibrinogen → no aggregation
76
how are the Hereditary Platelet Function Disorders diagnosed and treated?
- BS Syndrome: bleeding is intermittent and unpredictable, low platelets; treat with platelet transfusions and avoid anti-platelet meds
- GT: bleeding from minor cuts/trauma, treat with recombinant factor VII
77
what is the treatment for Hemophilia A?
Factor VIII replacement
- Recombinant or plasma-derived
78
what is the treatment for Hemophilia B?
Purified factor IX products or recombinant factor IX for treatment of minor and major bleeding
79
what is Congenital Hypercoagulability and how is it diagnosed?
- many patients with venous thromboembolism have inherited hypercoagulability
- No general screening test available: functional, immunologic, or DNA-based assays available for
antithrombin deficiency, protein C deficiency, protein S deficiency, APC resistance (factor V Leiden), and the prothrombin G20210A mutation
