Exam 3 Flashcards
(147 cards)
1
Q
Transferrin
A
Absorbed iron is carried by this
2
Q
Ferritin
A
Stores excess iron
3
Q
Patho/mechanism of disease of iron deficiency anemia
A
Blood loss, reproduction and growth, inadequate iron intake
4
Q
Symptoms of anemia’s
A
Fatigue
Tachycardia
Palpitations
Dyspnea or tachypnea on exertion
5
Q
Pica
A
Common in iron deficiency patients
Craving for specific food, even if it doesn’t contain iron
6
Q
Cheilitis
A
Irritation at corners of mouth
Seen in iron deficiency anemia
7
Q
Glossitis
A
Smooth red tongue, common in anemia’s
8
Q
Koilonychia
A
Spoon nails, common in iron deficiency anemia
9
Q
Plummer Vinson syndrome
A
Dysphagia due to esophageal webs, seen only in severe disease of iron deficiency anemia
10
Q
Lab eval for iron deficiency anemia
A
Microcytic, hypochromic
May see elevated RDW early in disease
11
Q
Ferritin levels in iron deficiency anemia
A
Decreased
**will be falsely elevated in inflammation
12
Q
B12 characteristics
A
Cobalamin Produced in microbes 100% needed from diet Stores well in tissues Takes years to deplete stores
13
Q
B12 deficiency anemia causes
A
Nutritional deficiency
Inadequate absorption
14
Q
Pernicious anemia
A
Large prevelance in patients 60+
Autoimmune destruction of gastric mucosa
Decreased instrinsive factor, causing severe b12 malabsorption
15
Q
General cobalamin metabolism
A
Made into transcobalalmin, goes into cells, bings to MMcoA, donates methyl to DNA RNA, proteins, etc.
16
Q
Why does b12 deficiency cause macrocytic anemia?
A
Accumulation of genetic material
17
Q
B12 deficiency anemia signs and symptoms
A
Can cause leukopenia/thrombocytopenia
Glossitis, anorexia, diarrhea
**neuropathy! Stocking/glove distribution
18
Q
B12 anemia lab testing
A
Low cobalamin
MMA and/or homocysteine levels elevated
Macrocytosis
19
Q
Folic acid deficiency general characteristics
A
Neural tube defects (spina bifida) in pregnancy, congenital heart disease
Abundant in citrus and leafy greens
2-3 month store
20
Q
Folic acid deficiency causes
A
Increased demand, dietary deficiency, drugs
21
Q
Folic acid deficiency anemia signs and symptoms
A
Similar to b12, often simultaneous
**no neural abnormalities!!!
22
Q
Lab testing for folic acid deficiency
A
Low serum folic acid
Cobalamin to rule out b12 deficiency
23
Q
Thalassemias pathophysiology
A
Defective globin synthesis
Leads to microcytic, hypochromic anemia
Asian, black, Mediterranean patients
24
Q
Alpha thalassemia pathophysiology
A
Gene deletion reduces alpha globin chain synthesis
25
Beta thalassemia pathophysiology
Point mutation, decrease or absent beta chain synthesis
26
How can you distinguish alpha and beta thalassemia?
Beta has basophilic stippling (also seen in lead poisoning)
27
Severe thalassemia, consider...
Splenectomy
28
Autoimmune hemolytic anemia
Autoantibodies form against RBC membrane causing hemolysis
IgG binds at body temp- autoimmune hemolytic anemia, warm auto Abs
IgM binds at cooler temps- cold agglutinin disease
29
Coombs test diagnoses...
Autoimmune hemolytic anemia
30
Sickle cell disease general characteristics
Autosomal recessive, amino acid substitution on beta globin chain
Hb S is unstable and prone to polymerization with other Hb S, leads to suckling
31
What are common triggers of sickling?
Hypoxemia and acidosis, deoxygenated Hb S
32
Heterozygous sickle cell...
Are typically hematologically normal
33
Homozygous sickle cell disease...
Causes the disease
34
Sickle cell disease diagnosis
Chronic hemolytic anemia
Sickle D cells visible on smear
Elevated acute phase reactants
Hemoglobin electrophoresis** confirmatory test
35
Sickle cell disease manifestations
Vaso-occlusive episodes/ crises
Acute chest syndrome- respiratory failure due to emboli, thrombosis, sometimes infection
36
Sickle cell and the spleen
Spleen is very active in removing old RBC and RBC fragments
Splenomegaly, splenic sequestration, can lead to hypocalcemia shock
Can cause auto splenectomy
37
Sickle cell disease traetment
Stem cell transplant for kids
**pain management
Hydroxyurea
38
Paroxysmal nocturnal hemoglobinuria General
Rare
Gene on X chromosome, but not X linked
Abnormal RBC vulnerability to complement (Coombs negative)
39
PNH symptoms/signs
Hemoglobinuria, jaundice, bone marrow suppression, increases at night
40
PNH labs
Flow cytometry, sorts and identifies cell types
**severe disease needs to be treated
41
Aplastic anemia
Injury or suppression of heme stem cell- marrow failure and inability to make mature blood cells
42
Hereditary Spherocytosis
RBC membrane protein defect, leads to hemolysis due to membrane fragility
43
Spherocytosis diagnosis
Osmotic fragility testing
Spherocytes in peripheral smear
44
G6PD deficiency
G6PD decreases oxidative stress, so a deficiency leads to denatured proteins
45
G6PD deficiency RBC presentation
Heinz bodies
RBC membrane damaged
Bite cells in peripheral smear
**enzyme assay confirms low G6PD
46
Anemia of chronic disease
Microcytic
Commonly from inflammation, organ failure, old age
47
Extrinsic pathway general
Triggered by tissue damage, started from exposure to tissue factor
Quick, small steps to form thrombin, positive feedback loop
Prothrombin time evaluates this pathway
48
Intrinsic pathway general
Trauma to blood or exposed collagen, less potent enough than extrinsic pathway
PTT evaluates this pathway
49
Plasminogen is activated to...
Plasmin, which does fibrin degredation
50
Platelet range indicative of platelet transfusion
<10-20 k
51
Petechiae
No blanching, erythematous or violaceous lesion
<3 mm
52
Purpura
3-10 mm
53
Ecchymosis
> 10 mm
54
Hemorrhagic bullae
Fluid filled lesion >10 mm
Vasculitis, necrotizing infections, etc
55
Thrombocytopenia in pregnancy
Often due to expanded plasma volume, HELLP
Hemolysis, elevated liver enzymes, low platelets
56
Pseudo thrombocytopenia
Platelet clumping can occur in lab testing, but citrated platelet count can verify true vs. pseudo thrombocytopenia
57
Immune Thrombocytopenia general
Dz of young women
Circulating antiplatelet autoantibodies, insufficient platelet production
Thrombopoietin levels low
58
Immune thrombocytopenia manifestations
Some asymptomatic
Mucocutaneous bleeds, mild nosebleeds
Some severe bleeds
59
ITP work up/treatment
Isolated thrombocytopenia on CBC
60
Thrombotic microangiopathy
2 forms, thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome
61
Thrombotic thrombocytopenic purpura general
Deficiency of ADAMTS13
Causes huge multimers and then thrombus formation
62
Thrombotic thrombocytopenic purpura manifestations
```
Classic Pentad-
Thrombocytopenia
Anemia
Fever
Neurological problems
Renal abnormalities
```
**rare
More likely to see thrombotic complications
63
Hemolytic uremic syndrome
Very similar to TTP, kids, endemic HUS usually caused by bacterial diarrhea
64
Heparin induced thrombocytopenia
IgG antibodies formed to heparin-platelet factor 4 complex
Usually asymptomatic, within 5-10 days starting heparin
65
Disseminated intravascular coagulation
Uncontrolled coagulation
Depletes the clotting system, causing lots!! Of bleeding and ischemic events
66
Disseminated intravascular coagulation related conditions
HELLP syndrome and sepsis
67
Hemoch- schonlein purpura
Systemic vasculitis in kids
Palpable purpura, arthritis, hematuria, maybe abdominal pain
68
Hemophilia A
Deficiency of factor VIII
69
Hemophilia B
Deficiency of factor IX
70
Genetics of hemophilia
X linked recessive, affects males and females will be carriers
71
Hemophilia clinically
Severe cases develop spontaneous hemarthroses and soft tissue bleeds
72
Hemophilia C
Factor XI deficiency
Mild bleeding risk, usually detected in surgery or trauma
Can supplement factor XI
73
Von willebrand disease general
Most common inherited bleeding disorder
Type 1- mild to moderate platelet type bleeding, epistaxis >10 and bleeding after dental work
Type 2,3- moderate to severe bleeding in childhood, may look like hemophilia
74
VWD diagnosis and treatment
Ristocetin cofactor activity
Type 1 traetment- DDAVP (des oppression)
75
Vitamin K deficiency
Vitamin K needed to activate 1971
76
Antiphospholipid antibody syndrome general
Autoantibodies to phospholipids (not SLE)
Phospholipids involved in much of the coag cascade
77
Anti phospholipid antibody syndrome symptoms
Venous or arterial conclusions, recurrent fetal loss
Need to rule out SLE
78
Factor V Leiden mutation
Most common inherited cause of hypercoagulability
Resistance to activated protein C, more common for DVT with homozygous
79
Suspect factor V Leiden mutation if....
Thrombosis on OCP
Cerebral vein thrombosis
DVT/PE in white patients
80
MGUS general
Asymptomatic premalignant clonal proliferation
Absence of end organ damage
Variable risk of progression
Non-igm MGUS
igm mgus
Light chain mgus
81
Abnormality on testing should prompt a ...
Bone marrow biopsy
82
When MGUS is found, what is the course of action?
WATCH, multiple myeloma is preceded by this.
83
MGUS complications
Fracture
Thromboembolism
Secondary cancer
84
MM general
Heme malignancy, stem cells that differentiated into plasma cells
In elderly
MGUS and end organ damage*
Malignant plasma cells can form tumors, bone dz means increased osteoclast activity
85
Bence jones proteins
In multiple myeloma, M proteins/globulin found in the urine
86
Amyloidosis
Found in multiple myeloma
Light chain fragments that aggregate into tangles, common cause of renal impairment and proteinuria
87
Multiple myeloma presentation
```
Anemia
Bone pain
Infection (pancytopenia)
Renal dz
Hyperviscosity
```
88
Multiple myeloma diagnosis
Pancytopenia
Anemia
**rouleaux, bundles of RBCs suggesting para proteins
Bone marrow biopsy to confirm dx
89
Smoldering myeloma
Meets hematologic criteria for multiple myeloma, but has no end organ damage
**just observe, like MGUS
90
Neutropenia
Absolute neutrophil count <1800
Greatest risk of infection if < 500
91
Congenital neutropenia
Genetic disruption of neutrophil development
High risk of infection, heme cancers in adolescence, early onset osteopenia and osteoporosis
92
Neutropenic fever
Hematologic emergency!!
Someone with neutropenia that develops a fever is treated very aggressively
93
Bone marrow transplant general
Allogenic-from an outside source
Recipient needs chemo before to clear out abnormal marrow cells
94
Myeloproliferative neoplasms general
Acquired clonal abnormality of heme stem cell
Shift into different types, but any can progress to acute myeloid leukemia
95
Polycythemia vera general
Overproduction of all 3 hematopoietic lines
EPO is low, yet RBCs still being produced
JAK2 mutation
96
Signs/symptoms of polycythemia vera
Hypervolemia/increased blood viscosity
Pruritus after a warm shower
Epistaxis
97
Polycythemia vera complications
Splenomegaly!!
Hallmark: elevated Hct
98
Primary myelofibrosis general
Fibrosis of the bone marrow, extramedullary hematopoiesis takes place in the liver, spleen and lymph nodes
JAK2 or MPL gene mutations common
99
Primary myelofibrosis presentation
Fatigue, splenomegaly, bleeding, bone pain
100
Primary myelofibrosis diagnosis
Anemia
Bone marrow biopsy is a dry tap or collagen
101
Essential thrombocytosis general
Proliferation of megakaryocytes and a rise in platelet counts
JAK 2 mutation
102
Essential thrombocytosis presentation
Asymptomatic thrombocytosis, splenomegaly, VTE
Rare: erythromelalgia (painful burning of hands with erythema)
103
Essential thrombocytosis bone marrow biopsy
Increased megakaryocytes
104
Myelodysplastic syndrome
Cluster of acquired clonal disorders of heme stem cells
Hyper or hypo cellular marrow, cell abnormalities
Preleukemia
105
Myelodysplastic syndrome presentation
>60 years old
Usually asymptomatic
Fatigue, infection, or bleeding
Splenomegaly
106
Paraneoplastic syndromes
Rare disorders triggered by an altered immune system response to a neoplasm
Symptoms that result from substances produced by the tumor or antibodies against cancer
107
What is leukemia?
Neoplastic disorders of white blood cells with the origin of the problem in the stem cell
Lymphoid or myeloid cell lines at any stage
As population of cells expands, bone marrow starts to fail
108
Acute lymphoblastic leukemia general
Lymphoblasts arrest in early development, then proliferate
Replaces normal marrow, circulating blasts, effects b and t cells
Most common childhood malignancy
109
Acute lymphoblastic leukemia manifestations
Fever without infection
Fatigue
Bone pain (marrow replacement)
110
Confirm acute lymphoblastic leukemia with...
Bone marrow biopsy with 20% or more blasts
Also, Ph chromosome means poor prognostic factor
111
Tumor lysis syndrome
Oncologic emergency!!
Metabolic abnormalities caused by release of intracellular contents
Renal failure, hypocalcemia, hyperkalemia, hyperuricemia, hyperphosphatemia
112
Chronic lymphoblastic leukemia general
Older patients, ~70
Incidentally discovered lymphocytosis, may be >100K
113
Chronic lymphoblastic leukemia presentation
Fatigue
LAD
HSM
Hypogammaglobulinemia
Low CD4 count, t cell deficiency
114
Hairy cell leukemia
Subtype of chronic lymphoblastic leukemia
Need:
Pancytopenia
Splenomegaly
Hair cells present on blood smear or BMbx
MEN around 50 years old
115
Acute myeloid leukemia general
Arrest of maturation in marrow cells, pancytopenia
20% or more blasts
116
Acute myeloid leukemia presentation
Related to cell deficiencies, like bleeding, fatigue and infection
Organ infiltration
Leukostasis
117
Acute myeloid leukemia pathognomonic finding
Auer rods in cytoplasm
Pancytopenia + blasts
118
Acute promyelocytic leukemia general
Differentiation stops at promyelocytes
Hyperacute but highly curable
119
Acute promyelocytic leukemia presentation
Presents like acute myeloid leukemia, but with coagulopathy
~40
120
Chronic myeloid leukemia general
Disorder of middle age
Hypermetabolic state, overproduction of white blood cells
121
Chronic myeloid leukemia presentation
Fatigue, night sweats and low grade fever
Splenomegaly
Myelocyte buldge
122
What is the myelocyte buldge?
Myelocytes outnumber more mature metamyelocytes in peripheral blood
Seen in chronic myeloid leukemia
123
What leukemia is prone to blast crisis?
Chronic myeloid leukemia
124
Leukemia cutis
Single or multiple nodules in the absence of systemic findings
Often asymptomatic lesions, but could have other signs of leukemia
125
Lymphoma general
Solid cancers of lymphocytes
126
Non hodgkin lymphoma general
Peak between 20-40 years
Mostly B cell
127
Non Hodgkin lymphoma presentation
Painless LAD, local or widespread
"B" symptoms
-fever, drenching night sweats, weight loss >10%
128
Non hodgkin lymphoma diagnosis
Lymph node biopsy
Peripheral blood often normal, CBC will NOT make diagnosis, need the biopsy
129
Indolent non hodgkin lymphoma
Follicular, marginal zone, little node thing
130
Waldenstroms macroglobulinemia
Monoclonal IgM paraprotein, low grade non hogdkin lymphoma
B cells that are hybrid of lymphocytes and plasma cells
131
Waldenstroms macroglobulinemia presentation
Rouleaux
Fatigue/anemia
Hyperviscosity
Cold agglutinin dz!
132
Aggressive non hodgkin lymphoma
Sezary syndrome, Burkitt lymphoma, cns lymphoma, peripheral t cell, mantle cell lymphoma
133
Non hodgkin lymphoma staging
Ann arbor staging
1- one node region
2- two+ node regions on same side of diaphragm
3- node regions on both sides of diaphragm
4- disseminated dz
134
Mycosis fungoides
Slowly progressive cutaneous t cell lymphoma
Can present just in skin for decades
135
Hodgkin lymphoma general
Reed stern berg cells! (Abnormal B cell precursor)
EBV most common cause
Usually localized to single group of nodes
136
Where do you see schistocytes?
Microangiopathic hemolytic anemia
**TTP and HUS
137
What is the cornerstone of therapy for TTP?
Plasmapheresis/plasma replacement
138
HIT is more common in what type of heparin?
Unfractionated
139
What is the diagnostic test for HIT?
ELISA
| Serotonin release assay**gold standard
140
Presentation of heparin induced thrombocytopenia
Usually asymptomatic
Thrombosis!
141
What substance is decreased in PNH?
Nitric oxide
142
What is the likely diagnosis if PNH causes bone marrow suppression?
Aplastic anemia
143
What 2 diseases that have mild bleeds can be treated with desmopressin?
Hemophilia A and wonvillebrand disease
144
What is the most common inherited thrombophilia?
Factor V Leiden mutation
145
What is the diagnostic test for MGUS?
SPEP
146
What are the two best tests for diagnosing leukemia?
Bone marrow biopsy and flow cytometry
147
Philadelphia chromosome is commonly found in...and sometimes in...
Chronic myeloid leukemia
Acute lymphoblastic leukemia
