Medical management of Hematologic Disorders Flashcards
(145 cards)
1
Q
What is polycythemia?
A
Body makes too many RBCs, making blood thicker than normal and causing blood 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 common causes of polycythemia?
A
-Obstructive sleep apnea
- Obesity hypoventilation syndrome
- COPD
- Testosterone replacement therapy
- Heavy cigarette smoking
- Fairly common in neonatal unit
4
Q
What are the 3 overall underlying causes of anemia?
A
- Decreased production of RBCs
- Blood loss
- Increased rate of destruction of circulating RBCs
5
Q
What are main symptoms of anemia?
A
- Fatigue
- Palpitations
- Shortness of breath
- Abdominal pain
- Bone pain
- Tingling of fingers and toes
- Muscle weakness
6
Q
What are the signs of anemia?
A
-Pallor
- Cracking, splitting, spooning of nails
- Jaundice
- Enlarged liver and spleen
- Lymphadenopathy
- Blood in stool
- Glossitis, smooth or red tongue, angular cheilitis
- Loss of taste
7
Q
What cause microcytic anemia?
A
- Iron deficiency
- Thalassemias
- lead poisoning
8
Q
What causes normocytic anemias?
A
- Hemolytic anemia
- Renal failure
- Blood loss
- Chronic disease
9
Q
What causes macrocytic anemias?
A
- B12 and folate deficiencies
- Hypothyroidism
10
Q
Describe causes of iron deficiency anemia
A
- Excessive blood loss (GI, menstruation)
- Poor iron intake (some countries, children)
- Poor iron absorption (Gastrectomy, GI)
- Increased demand for iron (pregnancy, chronic inflam)
11
Q
What are found in abnormal iron studies?
A
- Low serum ferritin
- Low serum iron
- High transferrin and Hgb iron binding capacity
12
Q
What is serum ferritin?
A
Bodys iron storage
13
Q
What is serum iron?
A
Amount of iron availavle
14
Q
What are treatments of iron def anemia?
A
- Look for source of bleeding and stop it
- Check lead in children
- Oral iron supplements
15
Q
Lead poisoning is the leading cause of what in children?
A
Cognitive deficits, peripheral neuropathy and renal disease
16
Q
What is the abnormal lead level in Phila?
A
3
17
Q
Lead Level >10
A
Phila Lead Health Homes Program–> Abetement and iron supplements
18
Q
Lead level >45 need
A
Chelation therapy
19
Q
Humans cannot synthesize what vitamin which is linked to anemia?
A
Vit B12
20
Q
Vitamin B12 and folate deficiency are needed for
A
RBC formation and growth
21
Q
Where do humans get Vita B12?
A
Animal products such as fish, poultry, meat, eggs and dairy. IS also found in fortified cereals and enriched milks
22
Q
Where do humans get folic acid?
A
Leafy green vegetables
23
Q
Vitamin B12 and folate deficiency causes ________ anemia
A
Macrocytic
24
Q
What is a cause of B12 deficiency?
A
Pernicious anemia (lack of intrinsic factor), malabsorption
25
Describe lab evaluation of B12 deficiency
- Check B12 and folate levels
- Eval specific metabolic intermediates which can accumulate in these deficiencies
- Schilling test
26
How do you treat B12 deficiency?
Supplement with Vit B12 (oral or IM cyanocobalamin; dietary)
27
What are causes of folate deficiency?
- Inadequate intake
- Increased demand
- Malabsorption
28
What are risk factors for folate def?
- Poor diet
- Alcoholism
- Hx of malabsorption disorders
- Pregnancy
29
What is treatment for folate deficiency?
Folic acid supplementation
30
What is the worst class of G6PD def?
Class I (severly deficient)
31
G6PD def causes toxic build up of oxidants. Precipitation leads to what?
Heinz bodies
32
How do RBCs use G6PD?
for carbohydrate metabolism
33
G6PD def is a type of
hemolytic anemia
34
How is G6PD triggered?
By infection, sulfa drugs and eating Fava beans or inhaling the pollen from the fava plants
35
What is the presentation of G6PD def?
Acute intravascular hemolysis- jaundice, palpitations, shortness of breath, dizziness
36
Sickle cell anemia- mutation in what chain results in amino acid substitution?
Beta globin chain
37
What causes the cells to sickle?
Hypoxia, decreased pH and dehydration
38
How does sickle cell anemia present?
- Acut painful crises
- Jaundice
- pallor
- Leg ulcers
- Organomegaly
- Heart failure
- stroke
- aseptic necrosis
- delays in growth
39
What are treatments for an acute crisis of sickle cell anemia?
- Rapid pain management
- IV hydration
- transfusion
- Antibiotics- Acute chest syndrome
- Oxygen
- Incentive spirometry
40
What are preventatives for sickle cell anemia?
- Hydroxyurea
- Long term, periodic blood transfusions
- Immunizations
- L-glutamine oral powder (Endaril)
- Hydration
- Iron chelation
- Folic acid supplementation and PCN
- Autologonous stem cell transplantation (Casgevy)
41
What does hydroxyurea do?
Increases total and fetal hemoglobin, also used to halt RBC, leukemia cells and platelets
42
What does L-glutamine oral powder (Endaril) do for sickle cell anemia?
Reduces oxidative stress
43
What are thalassemias?
Inherited blood disorder characterized by less hemoglobin and fewer RBCs in the body than normal
44
What causes the chain deformity in thalassemias?
Deletion of gene
45
What are symptoms of thalassemias?
Fatigue, weakness, pale or yellow skin, facial bone deformities, slow growth, abdominal swelling, dark urine
46
How are thalassemias diagnosed?
CBC, MCV (low 60s), iron levels, hemoglobin electrophoresis, retic, genetic testing
47
What are treatments for thalassemias?
Depends on severity. Severe forms may need blood transfusions, iron chelationa dn/or stem cell transplant
48
What is pancytopenia?
Bone marrow unable to produce RBC, WBC and platelets
49
How is aplastic anemia diagnosed?
Bone marrow biopsy (cells replaced with fat)
50
What are causes of aplastic anemia?
- Often unknown
- drugs (anti convulsants, bacterials, diabetic, diuretics, and sulfonamides, synthetic antithyroid drugs)
- Viruses (Hep, parvovirus (B19))
- Genetic (Fanconis)
- exposures (benzene and insecticides, radiation)
51
What are symptoms of aplastic anemia?
Weakness, fatigue, headaches, dyspnea with exertion, petechiae, ecchymoses, epistaxis, metorrhagia, gingival bleeding
52
What are treatments of aplastic anemia?
- Stop inciting cause if possible
- May resolve after hep B infection, Parvovirus B19 (red cell only)
- If mild (often no care or supportive care only)
- Severe (antibiotics, transfusions, GFs, stem cell transplant)
53
What is leukemia?
Cancer of WBCs; affects bone marrow and circulating blood
54
Who does AML primarily affect?
Adults (esp >50)
55
What is AML?
Neoplasm of myeloid (immature) WBCs
Uncontrolled proliferation in bone marrow space and subsequently appear in peripheral blood
56
What are prodrome symptoms of AML?
Flu like symptoms 4-6wks before diagnosis
57
What are nonspecific signs and symptoms of AML?
Fatigue, easy brusing, bone pain
58
AML can infiltrate and cause
Enlargement of tonsils, lymph nodes, spleen and gingiva and cause skin lesions
59
How is AML diagnosed?
Peripheral blood and bone marrow stained with Wright Giemsa
60
Who does ALL primarily affect?
Children
61
Which has more CNS effects- AML or ALL?
ALL
62
What is ALL?
Uncontrolled monoclonal proliferation of immature lymphoid cells in bone marrow and peripheral blood
63
Symptoms of ALL
(related to anemia, thrombocytopenia, fever and neutropenia)
Fever/infection, hemorrhage, organ enlargement, bone pain
64
How is ALL diagnosed?
Massive replacement of bone marrow space with leukemic blast cells
65
What are 3 phases of chemo for Acute leukemia?
- Induction
- Consolidation or intensification
- Complete remission/maintenance therapy
66
What is criteria for complete remission in acute leukemia?
- Platelet count > 100,000
- Neutrophil count >1000
- Bone marrow specimen with <5% blasts
67
What is consider "cured" for acute leukemia?
No leukemic cells
68
What is the most important sanctuary for ALL?
CNS
69
What are sanctuary sites for acute leukemia?
Leukemic cells can migrate to these areas where chemo agents cannot reach them
70
What is CML?
Neoplasm of mature myeloid WBCs
71
What neoplasm is the Philadelphia Chrom linked to?
CML
72
What is the mean age at diagnosis for CML?
67 Years
73
90% of CML patients are diagnosed durin
chronic phase
74
What symptoms of CML?
Night sweats, splenomegaly, fatigue, weakness, abdominal pain, abdominal fullness, weight loss.
Hyperviscosity of blood may cause stroke
75
What cells are increased during CML?
Basophils, eosinophils, neutrophils
76
What are CML medical managments?
- Interferon alpha or imatinib mesylate, inhibitor of tyrosine kinase, used in chronic phase
- Stem cell transplantation has resulted in remission in >70% at 10 yrs if treated before accelerate phase
77
What is CLL?
Neoplasm of mature clonal CD5+ B lymphocytes
78
What is the most common type of leukemia in adults?
CLL
79
What are symptoms of CLL?
Most asymptomatic
Fatigue, anorexia, weight loss, susceptible to infection, splenomegaly, lymphadenopathy, decreased serum immunoglobin levels
80
How is CLL diagnosed?
>5000 mature lymphocytes
and classified based on where lymph nodes are
81
What is the treatment for CLL?
No cure, treatment has little effect on survival times
82
What are lymphomas?
Cancer of the lymphoid organs and tissues that presents as discrete tissue masses
83
Initial signs of lymphomas
Often occur in mouth (Waldeyer's ring, tonsils, base of tongue, nasopharynx) and head and neck region
84
What are the types of lymphomas?
- Hodgkins
- Non-hodgkins
- Multiple myeloma
85
What is Hodgkin lymphoma?
Neoplasm of B lymphocytes
86
What contains characteristic tumor cell called Reed-Sternberg cell?
Hodgkin lymphoma?
87
What is the most common lymphoma in young adults?
Hodgkin lymphoma
88
What is the clinical presentation of hodgkin lymphoma?
- Painless mass or nontender enlarged lymph nodes
- 1/3 patients: fever, weight loss and night sweats
- Nodal biopsy or bone marrow aspirate
- large multinucleated Reed Sternberg cells
89
Does Hodgkin lymphoma have a high or low cure rate?
High
90
Flow of tx for Hodgkin lymphoma
Chemo --> Radiation --> Stem Cell transplant
91
What is Non-Hodgkin lymphoma?
Diverse group of malignant neoplasms of B cell or T cell origin (mostly B)
92
Clinical presentation of non-hodgkin lymphoma
- Enlarged painless lymph nodes, fever, weight loss
B symptoms (fever, night sweats, weight loss) indicate more aggressive clinical course
93
Diagnosis of Non-Hodgkin lymphoma
- Excisional biopsy of involved lymph node
- Staging of dz requires BCB, chemistry, chest X ray, CT scan and bone marrow biopsy
94
Describe Tx for follicular lymphoma
- Radiosensitive
- Watch and wait
95
Tx for diffuse large cell lymphoma
Chemo then radiation
96
Which involved Waldeyer's ring? Non-Hodgkin or Hodgkin?
Non-hodgkin
97
What is multiple myeloma?
Neoplastic proliferation of a single clone of plasma cells producing a monoclonal immunoglobulin.
Multiple tumors scattered throughout skeletal system
98
Median age or multiple myeloma?
69-71yrs
99
What is the clinical presentation for multiple myeloma?
- Multiple punched out lesions or mottled areas on radiograph
- Osteolytic lesions of jaw
- Amyloid deposition in various soft tissues
- Due to hypogammaglobulinemia, oneumonia, and pyelonephritis
100
What are signs and symptoms of multiple myeloma?
- Most common is persistent bone pain
- Anemia
- Weakness/fatigue, weight loss
- Hypercalcemia may cause headache, peripheral neuropathy
101
What are lab findings of multiple myeloma?
- Osteolytic bone lesions
- Elevated serum calcium
- Spike of immunoglobulins
- Abnormal Ig light chains in urine
- Anemia
- Neutropenia, thrombocytopenia
102
How is diagnosis of multiple myeloma confimred?
Protein electrophoresis of serum or urine.
-Presence of myeloma or monoclonal (M) protein band
- Most commonly detected is IgG then IgA and IgM
103
What is an autologous marrow graft?
Transplantation of the patient's own marrow, harvested earlier and set aside
104
Acute Graft versus host defense complications
Mucosal, skin, liver and GI involvement, intraoral mucosal ulcers and palatal mucoceles
105
Chronic graft versus host defense complications
Skin changes similar to those in scleroderma, sicca syndrome, malabsorption and features of autoimmunity
106
Drugs with "par" like heparin are
Anti-thrombin and on activated Factor X
107
Drugs with "tr" or "din" like Dabigatran are
anti-thrombin
108
Drugs with "xa" like Rivaroxaban are
Anti factor Xa
109
Clinical presentation of a bleeding disorder
petechiae and ecchymoses
110
What does PTT test for?
Intrinsic and common pathways
(best for coagulation disorders)
111
WHat does PT test for?
Extrinisc and common pathway
112
What does DIC results from?
When the clotting system is activated in all or a major part of the vascular system (associated with infection, obstetric complications, cancer and snakebites)
113
In DIC, what is the clinical problem?
Bleeding, not thrombosis
114
Acute DIC clinical presentation
Severe bleeding from small wounds, purpura and spontaneous bleeding from the nose, gums, GI tract or urinary tract
115
Chronic DIC clinical pres
thrombosis more common than bleeding
116
Diagnosis of DIC
- Prolonges PT, PTT and thrombin time
- Thrombocytopenia (consumption of platelets)
- Secondary fibrinolysis generated increased titers of FDPs, mesaured by latex agglutination or D dimer assays
- Chronic DIC difficult to diagnose
117
What is tx of DIC?
- Try to reverse the cause and control major symptoms
- Replace consumed coal factors; restore fibringoen levels
- Cryoprecipitate if bleeding is major
- Fresh frozen plasma
- IV heparin if thrombosis is problem
- Long term heparin infusion for prophy
118
What is thrombosis?
Formation, from components of blood, of an abnormal mass within the vascular system
119
What are major factors of thrombosis?
- Injury to vessel wall
- Alterations in blood flow
- Changes in composition of blood
120
What are causes of venous thrombosis?
Age, history, immobilization, obesity, infection, hospitilization, major surgery and pregnancy
121
What are causes of both venous and arterial thrombosis?
Malignancy, hormonal therapy and DIC
122
What are causes of arterial thrombosis?
Atherosclerosis
123
What are treatments for arterial thrombi?
Agnets with antithrombin and antiplatelet activity
124
What are symptoms of venous thrombi?
Drugs that prevent thrombin formation or lyse fibrin clots
125
What are examples of direct thrombin inhibitors?
"din" or "tr"
Lepirudin, desridin, argatroban, hirudins, pradaxa
126
Clinical presentation of coagulation disorders
Ecchymosis, hemarhrosis, dissecting hematomas
127
What is the clinical pres for platelet disorders?
Petechiae and ecchymosis
128
What is the clinical pres for vascular defects?
Petechiae, bleeding
129
What is Hereditary hemorrhagic telandiectasia?
- Osler-weber rendu syndrome
- Rare autosomal dominant disorder
- Characterized by multiple telangiectatic lesions involving skin, mucous membranes and viscera
130
HHT treatments
- Laser tx for cutaneous lesions
- Split thickness skin grafting
- Embolization of arteriovenous communications
- Hormonal therapy for epistaxis
- Pulmonary resection or embolization for pulmonary arterivenous malformations
- Hormonal therapy and laser coag for GI lesions
131
What is vWF disease?
Inherited defect of platelet adhesion
132
What does vWF do?
Binds factor VIII, protects from destruction
133
What is clinical pres of vWF?
- Mild: cutanous and mucosal bleeding
- Severe: hemarhroses, dissecting intramuscular hematoma
- GI bleeding, epistaxis, menorrhagia common
- May have personal or family hx
134
Lab results for vWF
- Prolonged PTT
- Normal PT, platelet count, thrombin time
135
What is tx of vWF?
- Cryoprecipitate
- Desmopressin (VP analogue that increases factor VIII activity) IV or nasally 1 hr before surgery
136
What is Bernard-Soulier syndrome?
Platelets are large and defective; unable to interact with vWF
137
Bernard-Soulier symptoms
- Epistaxis, easy bruisingm mucous membrane bleeding, preoperative bleeding, menorrhagia
Many have ecchymosis, gingival bleeding and GI bleeding
138
Lab tests for Bernard-SOulier
Low platelet count, large platelets, faulty platelet adhesion, poor aggregation with ristocetin
139
Treatment for Bernard-Soulier
Platelet transfusions when absolutely necessary and avoidance of antiplatelt medications
140
What is Glanzmann's thrombasthemia?
Rare autosomal recessive disease of platelet dysfunction; platelts cannot bind fibrinogen and aggregation does not occur.
Bleesing is unpredictable
141
What are symptoms of Glanzmann's thrombasthenia?
- Epistaxis, easy bruiding, oral and gingival hemorrhage, GI bleeding, perioperative bleeding, hemarhrosis and menorrhagia
142
Lab test for Glanzmanns thrombasthenia
low platelet count, large platelets, faulty platelet adhesion, poor aggregation with ristocetin
143
Tx for Glanzmanns thrombasthenia
Lifestyle advice and education, local measures, antifibrinolytic agnts, hormone tx, platelt transfusions and recombinant factor VII to control bleeding
144
What is hemophilia A a def of?
Facotr VIII
145
Management for Hemophilia A
Factor VIII replacement (recombinant or plasma derived)
