Heme Flashcards
(149 cards)
1
Q
what are the causes of bone marrow failure?
A
- Nutritional deficiency: Vitamin B12, Folate, Iron
- Marrow toxins (myelosuppressive): Drugs (many), chemotherapy, radiation, ethanol
- Infections: HIV, HBV, HCV, EBV, CMV, Parvovirus B19- common childhood infection
- Marrow replacement: Infections (fungal, TB), sarcoid, leukemia, lymphoma, myeloma, metastatic carcinoma
- Autoimmune diseases
- Primary bone marrow diseases: Fanconi anemia, paroxysmal nocturnal hemoglobinuria, many others
2
Q
3 requirements for hematopoiesis
A
- Healthy Bone Marrow
- Hormones/ Cytokines (stimulus—foot on gas pedal)
-Erythropoietin- hormone from the kidney that stimulates marrow to produce RBCs, anemia from chronic renal failure is due to lack of Erythropoietin
− Thrombopoietin- hormone from the liver that stimulates marrow to produce RBCs
-Growth Factors- hormone that stimulates marrow to produce WBCs (gray cell) - Nutritional Factors- ( gas tank)
Iron (RBCs), Folate, Vitamin B12 (RBC, WBC, and platelets)
3
Q
only cell without nucleus
A
rbc
4
Q
cell whose nucleus divides a bunch then cytoplasm splits
A
megakaryocytes to platelets
5
Q
which blood cell develops within its own lineage?
A
lymphocytes
6
Q
which part of bone marrow is lined with endothelial cells and fibroblasts support the development of blood cells (not blood making tissue)
A
stroma
7
Q
primary hematopoietic organ in fetus
A
liver
8
Q
which organs can make blood if bone marrow diseased?
A
liver and spleen
9
Q
what’s primary hematopoietic organ at birth?
A
bone marrow
10
Q
what 2 things compromise blood?
A
formed elements (RBC,WBC, platelets) and plasma
11
Q
whats in plasma?
A
- Water: main component
- Dissolved ions: Na+, K+, Cl-, H+, Mg++, Ca++
- Proteins (about 3 dozen): Carrier Proteins (Albumin (Uhaul van of drug system), Lipoproteins, Transferrin, Many others), Immunoproteins (IgG, IgA, IgM, IgD, IgE, Complement proteins), Coagulation proteins
12
Q
which blood cell is only one that returns to BM?
A
lymphocytes
13
Q
mechanisms of cytopenias
A
- Decreased Production (6 categories from below—sick bone marrow)
- Increased destruction/consumption
- Sequestration/spleen
14
Q
what produces epo? can you measure epo with creatinine or kidney function tests?
A
kidney; no you can’t measure it that way because epo production via kidney is a completely separate function form how well it can clear stuff
15
Q
what’s the test that can differentiate between destruction or production problem of RBCs, first test for evaluating anemia?
A
reticulocyte count
16
Q
is a rx necessary for blood products?
A
yes
17
Q
indications for fresh frozen plasma
A
• Indication: coagulation deficiencies
− Liver failure
− Disseminated intravascular coagulopathy (DIC)- all coagulation factors are consumed?
− Vitamin K deficiency- Vitamin K-dependent factors (II, VII, IX, X) (remember Warfarin inhibits this)
− Warfarin toxicity
− Massive blood loss
18
Q
indications for platelets transfusin
A
• Indications: Thrombocytopenia (
19
Q
how many platelets are in one unit from one donor? how many of those are usually pooled together for the patient?
A
10-15K; pool 6-8 people’s together to give them 60k
20
Q
indications for pRBCs
A
− Hemodynamically unstable: P>100/min, RR>30/min, hypotensive, decrease O2 sats, dizziness, weakness, angina, and altered mental status
− Leukemic processes, hemolytic anemia, other anemias, surgical or traumatic blood loss can decrease RBCs
• Criteria: usually Hgb of
21
Q
indications for albumin
A
• Indication: equalize the intra and extravascular osmotic pressure
− Situations of hypovolemia and hypoproteinemia
− Burn injury
22
Q
percent Rh+ in population
A
70%
23
Q
if someone is Rh- and you give them Rh+ blood what will happen?
A
they will not react much at first but will mount an immune response and develop anti-D ab and will attack Rh+ blood next time
24
Q
what is the difference between type and screen and type and crossmatch?
A
type and crossmatch includes combining donor and recipients blood cells to look for agglutination
25
who is a universal donor (for RBCs)? for plasma?
o negative (no antigens for pt to attack); for plasma: AB (no antibodies to attack patients own blood)
26
clinical presentation of hemolytic transfusion reaction and tx?
patients blood attacks transfused blood
Clinical presentations: DIC, Acute Renal Failure, Acute Tubular Necrosis, Shock
− Triad of Fever, Flank pain and red/brown urine
tx: Medical Emergency- STOP TRANSFUSION immediately, Maintain airway, start Saline at 100-200ml/hr. Notify the blood bank immediately
− Obtain blood and urine samples.
− Vasopressors (Dopamine) indicated for hypotensive patients.
27
febrilel nonhemolytic transfusion rxns: what is it? clinical presentation, tx
interleukins and TNF alpha in RBCs that attack patients cells
Fever, Chills, Rigors, Mild Dyspnea
tx: tylenol or benadryl
28
delayed hemolytic transfusion rxn: defintion, clinical presetnation, tx
atpical ab in recipients blood that occurs 2-10 days later, mild presentation of Slight fever, falling hematocrit, mild increase of unconjugated bilirubin and spherocytes in blood smears
no tx necessary
29
can you get an anaphylactic transfusion rxn from blood?tx
yes: same presentation as other anaphylaxis: shock, hypotension, angioedema, respiratory distress
tx: stop transfusion, give epic
30
uritcarial transfusion rxn:
blood products given cause histamine release of hives or urticaria, not necessary to stop transfusion, can give bendadryl
31
post transfusion purpura; tx? can they get a platelet transfusion in the future?
sensitized to antigen on platlets; severe thrombocytopenia lasting days to weeks, usu 5-10 days after transfusion.
tx with corticosteroids (blunt immune response) can get platelets later but they should be washed first
32
what kind of rxn? sudden onset of respiratory distress during or after transfusion − Fever, tachypnea, tachycardia, and hypotension can occur. how do you tx?
immune response in pulmonary vasculature
| • Treatment: Supportive. Mechanical Ventilation is required in 72% of the patients in one study.
33
blood product complications
```
fluid overload
rxn to preservatives in it
coag defects d/t to massive transfusion and dilution
chelation of Ca2+
hyperkalemia
hypothermia (blood stored in cold)
iron overload
air embolism
citrate toxicity (anticoagulant)
```
34
which pregnancy would be most problematic for an Rh- mother without med intervention?
second one, In the first pregnancy, a mother’s Rh- blood will not attack Rh+ babies blood because she hasn’t developed antibodies against it yet
However, during delivery or any trauma, the babies blood can mix with hers and the mother can develop the anti-D antibodies
During her second pregnancy her anti-D Ab could cross the placenta and attack the babies blood
35
what med is given to prevent Rh- negative mother from attacking Rh+ baby?
rhogam: attaches to fetus’s Rh+ antigen, preventing the mother’s immune response
36
indications for rhogam
• all Rh- women: 1 dose @ 26-28 weeks gestation (early in 3rd trimester).
• Spontaneous/Induced Abortion >12 weeks gestation. (before that baby has not formed Ab yet)
• Ectopic pregnancy.
• Multifetal reduction.
• Invasive procedures: e.g. Amniocentesis.
• Threatened abortion.
• Fetal death in 2nd or 3rd trimester.
• Blunt trauma to the abdomen.
• Antepartum hemorrhage in 2nd or 3rd trimester.
-and after delivery
37
what is the largest lymphoid organ?
spleen
38
when should you biopsy the spleen?
NEVER. not done anymore b/c it can pop. just take it out if concerned.
39
why would you take out a spleen?
* Immune cytopenias
* Hematologic malignancy
* RBC membrane/enzyme disorders
* Thalassemia
* TTP
40
someone has elevated WBCs and platelets, what should you consider?
that their spleen is taken out
41
how do you prevent post splenectomy sepsis?
vaccinate! h. flu, n. meningitidis, s. pneumo
42
why don't we give splenic kids prophylactic abx ?
resistance
43
how do you manage infections in asplenic people?
they need an rx of abx to take, even if its seems viral, and take it then proceed to nearest healthcare facility
44
− To find, ingest, and kill invading microorganisms
− Bactericidal activity is mediated by production of hydrogen peroxide, superoxide, hydroxyl radicals, hypochlorous acid, nitric oxide
neutrophils
45
causes of increased and decreased neuttrophils
* **Increase Neutrophils: bacterial infection, physiologic stress, corticosteroids
* **Decrease Neutrophils: viral infection, drugs/toxins, some bacterial infections (Brucella)
46
• Secrete cytokines that induce fever and inflammation
| Increase occurs with chronic infections (TB), lymphomas, and granulomatous diseases (sarcoid)
monocytes
47
what WBC is increased?
− Chronic inflammatory skin disorders (like bad eczema)
− Invasive parasitic diseases (checked when immigrants arrive)
− Hypersensitivity states (allergies, vasculitis)
− Certain malignancies (Hodgkin’s disease
eosinophilia
48
what causes basophilia?
occurs in allergic disorders and myeloproliferative diseases (CML, Polycythemia vera)
• After leaving the circulation, basophils differentiate into tissue mast cells
49
how do basophils become mast cells that release histamines?
Membranes contain high affinity IgE receptors, Ig antibodies to environmental antigens that cause allergies
• **Binding of antigen / IgE complex to basophil membrane causes degranulation and release of substances (e.g. histamine) with powerful vasoactive and inflammatory properties
50
• Increased in most viral infections
lymphocytes
51
causes of leukocytosis: reactive
* Bacterial (neutrophils)/Viral/ Parasitic (eosinophilia) infection
* Inflammation (eosinophils)
* Allergy (eosinophils)
* Physiologic stress
* Drug induced
* Asplenia
52
causes of leukcytosis: malignant:
* Hematologic
| * Non hematologic
53
practical # of WBCs (low and high) to call hematology
50000
54
causes of leukopenia: decreased production and destruction
```
a) Decreased production
• B12 / Folate deficiency
• Marrow aplasia / dysplasia
• Marrow replacement (tumor, infection)
• Marrow damage (drugs, toxins, radiation)
• Autoimmune
b) Increased destruction / consumption
• Hypersplenism
• Autoimmune
• Overwhelming infection / sepsis
```
55
factors required for blood cell synthesis?
healthy bone marrow, "gas" nutrients like iron, b12, folic acid, gas pedal, foot on gas pedal=epo
56
best place to tell if someone is pale/anemic
palpebral conjunctiva
57
sx of anemia
* Fatigue; ↓ stamina with exercise
* Dyspnea
* Tachycardia
* Dizziness; lightheadedness
* Fainting
* Headache
58
important PMH questions for anemia
* Hx of anemia?
* Previous treatment and response
* Transfusion history
* Menstrual history, abnormal bleeding
* Pregnancy (frequency)
* Bariatric surgery
* Medications:
* NSAIDS
* Anticoagulants
* Myelosuppressive drugs
59
fm qxs in anemia
* Ethnic background
* Sickle Cell Anemia
* Thalassemia
* Presence of anemia in first degree relative
* Age of diagnosis
* Severity
* Jaundice
* Cholecystectomy
* Splenectomy
60
soc hx qxs in anemia
* Dietary Habits
* Iron, B-12, folic acid
* Vegetarian eating patterns
* Alcohol Consumption
* Folic acid- decreases absorption; interferes with conversion to active form and release
* B-12- decreases absorption
* G.I. bleeding
* Immune suppression
* Liver disease (independent of vit def)
* Impaired biosynthetic processes
• Blood Donations
61
anemia PE
• Pallor (skin, oral mucosa, conjunctiva, nail beds, palms); ecchymosis, petechiae
• Jaundice, hepatosplenomegaly (hemolytic anemia)
• Tachypnea, tachycardia; systolic flow murmur
• Edema
• Lymphadenopathy
➢ Stool occult blood
62
normal hgb ranges for males and females
Adult male = 14.4-16.1 g/dL Adult female = 12.2-14.7 g/dL
63
classification of anemia
10-12 g/dL
64
anemia and high reticulocyte count=?
RBCs being destroyed
65
low absolute reticulocyte count means?
decreased BM production
66
normal absolute reticulocyte count
20000-75,000/microliter
67
anemia + small red blood cells=?
iron deficiency anemia, thalassemia, lead poisoning
68
anemia + big red blood cells?
* ***B-12/folic acid deficiency
* Pernicious Anemia
* Liver disease
* Hypothyroid
* Drugs (e.g.TMP/SMX, Metformin, Hydrea)
* Myelodysplastic syndrome
69
lab test to help you determine size of RBCs (besides smear); what's low, what's high?
mean corpuscular volume MCV; low 100 think B12 or folate deficiency
70
causes of normocytic anemia
```
think anemia of chronic disease: •
ACD/AI (most)
• Renal Failure
• Early iron deficiency (acute bleeding or intermittent blood loss)
• CLL, CML, Aplastic Anemia
```
71
anisocytosis
large variations in size of RBCs on RBC distribution width
72
hypochromic vs. normochromic
hypochromic=low hemoglobin concentration in RBCs=pale centers
73
total iron binding capacity should increase when?
total serum iron is low
74
tx of anemia d/t to acute blood loss
volume expansion, transfusion
75
tx of anemia d/t chronic blood loss
depends on seveirty, transfusions, oral/parenteral iron supplement
76
lab workup for someone who is iron deficient?
Diagnosis
| • ↓ Hgb (
77
what's a common cause of anemia worldwide?
parasites
78
Pica
craving of non food items that is a sx of iron deficiency
79
ddx of anemia
* Anemia of Chronic Disease/ Anemia of Inflammation
* Thalassemia
* Sideroblastic Anemia
* Myelodysplastic syndrome
* Lead toxicity
80
anemia: low TIBC, normal ferritin, normocytic, normochromic
anemia of chronic disease
81
causes of B12 deficiency anemia
low dietary intake (rare), malabsorption, pernicious anemia
82
neuro sx of B12 deficiency
* Peripheral neuropathy
* Parasthesias
* ↓ vibratory and proprioception
* Ataxia
* Weakness, inability to walk
* Dementia
* Psychological disturbances
* Sub-acute combined systemic degeneration (irreversible)
83
```
• ↓ Hgb and HCT
• ↑ MCV (usually > 100)
• Low reticulocyte count
• Pancytopenia (severe)
what kind of anemia?
```
usu B12 deficiency or folate deficiency, check serum B12 or antibodies for pernicious anemia
84
causes of folic acid deficiency anemia
• Poor Dietary Intake (top 5 nutrient deficiencies → fortification):
Risks → elderly, diets lacking green, leafy vegetables, anorexia, and cancer
• Increased/Impaired Utilization
Risks → Premature infants, ETOH, hemolytic anemia, dialysis, ICU patients
Pregnancy and Lactation (additional 400 ug)
Anti-folate Drugs
• Impaired Absorption → gastric resection, ileal disease, alcohol abuse (also utilization and storage)
85
if 3 blood cells lines are off you need a ____
bone marrow bx
86
triad of hemolytic anemia
* Normocytic or microcytic anemia
* ↑ reticulocyte count
* Hyperbilirubinemia (indirect)
87
first 2 tests for hemolytic anemia
CBC with diff, reticulocyte count
88
identifying and treating hereditary spherocytosis:
Child with:
Anemia
Jaundice
Splenomegaly
Labs:
Low hgb
High retic
MCV (N or slightly L)
MCH and MCHC usually elevated and combined with high RDW suggests diagnosis (sensitivity of 63% and specificity of 100%)
tx: Control anemia (transfusions)--if affecting growth
Folic acid for chronic red cell production?
Splenectomy may be required to control
Cholecystectomy if gallstones form
89
indications for splenectomy
#1 to treat a disease in which blood cells are destroyed in the spleen
#2 to prevent increasing splenomegaly and hypersplenism
90
hemolytic anemia: pyruvate kinase deficiency
| presentation, pathophys, dx, tx
missing glycolytic enzyme, cell has no energy, rigid=hemolyzed
presentation: decreased hgb, neonatal jaundice, increased retakes, abnormal cells on smear, enzyme test +
dx: enzyme test
tx: folic acid b/ high cell turnover, avoid transfusions and iron
91
```
structural hemolytic anemia: G6PD deficiency
pathophys
presentation
dx
tx
```
pathophys: x-linked, missing G6PD, can't respond to oxidative stress, spleen removes them
presentation: CBC normal between episodes of stress, stress=drugs, fava beans, infection;
sx: malaise, weakness, abdominal or back pain, increased bilirubin, jaundice, dark urine/ hemoglobinuria
dx: normochromic, normocytic, during attack decreased hgb, increased relics, G6PD screening
tx: avoid offending agent, +/- transfusions in acute episodes
92
microcytic anemia, increased reticulocyte counts, imbalance of alpha and beta chain global production, mutations in 1-4 of alpha genes
alpha thalaseemia
93
beta-thalassemia major presentation
in first or 2nd year when hgb F production stops, severe anemia requiring regular transfusion, facial abnormalities
94
labs in alpha and beta thalassemia
```
Mild to severe anemia
decreased Hgb and HCT
decreased MCV (microcytic)
decreased MCH (hypo chromic)
increased reticulocytes
Hgb Electrophoresis
Genetic mapping
```
95
anemia with high reticulocytes and vaso occlusive episodes
sickle cell anemia
96
tx of sickle cell
hydroxyurea: increases Hgb F, transfusions, folic acid, iron chelation if transfused, tx infections promptly
97
pt walks in with low hgb, increased relics, no inherited deficiencies, what tests to do?
ask yourself: how low is hgb? past hgb levels for comparison? otherwise healthy? check LDH, bilirubin, and coombs
98
patient with anemia, jaundice, splenomegaly, +/- syncope or HF
Positive Direct Coombs
Low hgb, high retic, high LDH, high indirect bili
warm antibody AIHA: igG attaches to RBCs at 37, stop offending drug give corticosteroids ASAP
99
```
patient with anemia, Acrocyanosis in fingers, feet, nose, and earlobes due to intra-arteriolar agglutination
Mild anemia
Jaundice, splenomegaly
RBCs clumping on smear
Fever
More common in elderly
```
cold antibody AIHA
100
secondary causes of warm antibody AIHA
```
Connective tissues disorders (SLE, RA)
Lymphoma, CLL
Carcinoma
Drugs-penicillin type, quinine, quinidine (etc.)
Post-viral (children)
```
101
secondary causes of cold antibody AIHA
```
Lymphoprolipherative disorders (NHL, CLL)
Infections (infectious mono, micoplasmal pneumonia, EBV, CMV)
```
102
what are causes of + indirect coombs?
Hemolytic disease of the newborn
Hemolytic transfusion reactions
Allogenic BMT, liver, renal, and heart transplant
103
what is a cause of coombs destruction of anemia?
mechanical shearing of RBCs
104
name of a structural anemia that is a miler form of hereditary spherocytosis
hereditary elliptocytosis
105
drugs that aggravate G6PD deficiency
antimalarials, nitrofurantoin, NSAIDS, quinidine, quinine, sulfonamides, aspirin
106
normal fetal hgb type; adult type
fetal: alpha2 gamm2; adult: alpha2beta2
107
test to look at hemoglobin type
hemoglobin electrophoresis
108
four steps of blood clotting
primary hemostasis (platelets), secondary hemostasis (coagulation cascade) hemostasis stops, fibrinolysis (clot dissolves)
109
most common inherited bleeding disorder, males and females affected
von Willebrand dz
110
signs of a primary hemostatic defect
prolonged initial bleeding, petechiae, mucosal bleeding, bruising
111
signs of a secondary hemostatic defec
hemarthrosis, ecchymosis, soft tissue bleeding
112
labs and sx in von willebrand
| labs: what will their iNR, aPTT, platelets look like? what other lab tests can you do to dx this disease?
```
sx: primary problem: Epistaxis, dental bleeding, bruising, menorrhagia (initial bleeding after challenges)
labs:
• Normal INR
• Normal or mildly prolonged aPTT (if Factor VIII low)
• Normal platelet count
• Abnormal PFA-100 (PFCT)
• ↓von Willebrand antigen
• Low factor VIII
• Low ristocetin cofactor activity
```
113
tx of von willebrand
avoid aspirin, DDAVP (desmopressin in inhaled form that releases VWF), amicar and lysteda (slows down clot dissolving), plasma derived factor VIII concentration)
114
what are these clinical features indicative of? joint bleeds, esp around knee, soft tissue bleeds,
• Normal INR
• Markedly prolonged PTT
• Normal platelet count and function
hemophilia
115
almost all people with DVT or thombophilia have triggers + ____?
genetic predisposition
116
elevated levels of d-dimer may also be seen in?
• Elevated levels may indicate clot, also be seen with: cancer, infection, trauma, surgery, pregnancy, inflammation
117
what are the two inherited mechanisms of thrombophilia? what factors are involved in each?
1. Failure to control thrombin generation- Factor V Leiden, Prothrombin Gene Mutation, Protein C deficiency, Protein S deficiency
2. Imparied neutralization of thrombin- Antithrombin-III deficiency
118
Are mutations of Protein C, S, and Antithrombin more common? or are mutations of Factor V Leiden and prothrombin?
Factor V Leidin and prothrombin are more common
119
when to screen for hereditary thrombophilias
• Young age at diagnosis of first clot (
120
what are some acquired conditions that can cause thrombophilia?
```
Conditions
• Antiphospholipid antibodies
• Malignancy
• Inflammatory Bowel Disease
• Nephrotic syndrome
• Myeloproliferative Disorders
• PNH
• CHF
• Sepsis / DIC
• HIT
```
121
acquired triggers that can cause thrombophilia
Immobilization
• Surgery
• Trauma
• Pregnancy / Postpartum- increases thrombosis risk
• Estrogens (OCPs, HRT)- 3-4 fold increased risk
• Older Age
122
how should you manage asymptomatic carriers of thombophilia?
prophhlax at times of risk, avoid OCPs, hormone replacement therapy, pay attention to FH
123
who should get indefinite anticoagulation therapy?
* Unprovoked event
* Recurrent event
* Life-threatening event
* Factor V Leiden homozygote
* Combined thrombophilias
* Persistent antiphospholipid antibodies
124
how are platelets produced?
megakaryocytes: endomitosis, nuceli keeps dividing and cytoplasm doubles in size then eventually gets too big and stuffs off "platelets" (part of the cytoplasm), each produces 2000-3000 platelets
125
what important parts of platelet structure are there in the membrane and the inside?
1. Membrane
• Phospholipids- provide a surface for coagulation cascade
• Glycoproteins
− GP Ia/IIa: collagen receptor - platelet adhesion when collagen exposed
− GP Ib/IX/V: vonWillebrand factor receptor - platelet adhesion
− GP IIb/IIIa: fibrinogen receptor - platelet aggregation
2. Granules
• Dense granules: contain ADP, Ca++, and serotonin which contribute to platelet aggregation and coagulant activity
• Alpha granules: contain a variety of proteins which contribute to platelet adhesion, aggregation, and coagulant activity (e.g. factor V, vonWillebrand factor, fibrinogen, platelet factor 4)
126
mechanisms of thombocytopenia
decreased production, increased destruction, sequestration (hypersplenism)
127
characteristics of drug induced thrombocytopenia
myelosuppression or immunologic, rapid onset, stop offending drug!
128
clinical features of HIT? how to tx it?
• Low platelets, extreme bruising, microarterial thrombi from antibody attacking heparin/platelet com
STOP all heparin, consult heme
it is a clinical dx because labs to confirm can take too long
129
ITP:
definition
clinical features
immune thrombocytopenic purpura
autoantibodies bind to platelets and accelerate splenic clearance
```
clinical features:
symptoms for months
• Petechiae in dependent regions
• Ecchymoses
• Menorrhagia, epistaxis, gingival bleeding- mucosal bleeding
• Otherwise healthy
• No lymphadenopathy or splenomegaly
labs: really low #, large platelets, nml coag times, increased megakaryocytes in marrow
```
130
ddx of ITP
drug induced, HUS (hemolytic uremic syndrome), Aplastic anemia/leukemia (usu >1 sx), inherited platelet production abnormalities, sepsis, DIC, splenomegaly, ALPS, immune def.
131
DIC
defintion
clinical features
tx
disseminated intravascular coagulation
excessive activation of coagulation systems usu after infection or in malignancy
clinical features:
• Microvascular thrombi cause widespread tissue ischemia, multiple organ dysfunction, and death
• Bleeding due to consumption of platelets and plasma clotting factors
• Thrombocytopenia
• Prolonged coagulation times (PT/INR, PTT)
• Low fibrinogen
• Schistocytes on blood smear- RBCs ripped apart in circulation
tx: tx underlying cause, consider heparin, transfuse platelets
132
TTP definition, clinical features, tx
```
definitio: endothelial injury, vonWillebrand factor cleaving protease, and systemic platelet aggregation
classic pentad of clinical features:
1. Thrombocytopenia
2. Microangiopathic hemolytic anemia
3. Neurologic symptoms- ↓blood flow to brain
4. Renal dysfunction
5. Fever
tx: plasma exchange
```
133
```
would these conditions be an indication for allo or auto HSCT?
• Lymphomas
• Multiple myeloma (not curative!)
• APL (acute promyelocytic leukemia)
• Germ cell tumors
• Ovarian cancer
• Neuroblastoma
• Autoimmune disorders
• Amyloidosis
```
auto
134
indications for allo transplant
* AML, ALL
* CML, CLL if failed tx
* MDS
* MPD
* Relapsed multiple myeloma or lymphoma post auto xplant
* Aplastic, fanconi, sickle cell, blackfan-Diamond anemias (uncommon)
* Peds: errors in metabolism, epidermolysis bullosa, congenital immune deficiencies
135
which is highest way of transmitting gHIV?
vertical (mother to child)
136
what component does HIV need to get into a cell?
a cd4 receptor
137
HIV life cycle
enters blood, attaches to CD4 cell, reverse transcriptase to DNA, integration in hosts DNA, replication, vision assembly, budding
138
by what day of infection is hIV replicating?
day 4-11
139
do retroviruses carry genetic info as DNA or RNA?
RNA then reverse transcript it to DNA and use host's machinery
140
Ryan White Care Act
(anyone can receive coverage for tx for HIV/AIDS)
141
can you eradicate HIV?
NO
142
how does hIV progress to AIDS?
. CD4+ T-cell count below 200 cells/μL
2. CD4+ T-cell percentage of total lymphocytes of less than 15%
3. Or one of the defining illnesses.
143
ELISA test is + for HIV, what is next step?
western blot confirmatory test
144
how long does it take following infection for hIV test to be positive?
22-27 days
145
who should get tested for hIV?
* Anyone requesting it, regardless of risk
* Anyone with high risk behavior: MSM, IVDU, 1+ partners, exchanged sex for $ or drugs
* Certain medical conditions: AIDS defining illness, oral candidiasis, generalized unexplained LAD, STIs, TB, pregnancy, pneumonias, recurrent vulvar candidiasis, herpes zoster, key is recurrent
* Anyone who has been sexually assaulted
* Anyone with occupational exposure
146
who should get screened for HIV?
routine for everyone 13-64
| repeat for those at high risk
147
most common sx of HIV
* Fever (96%)
* LAD (74%)
* Pharyngitis (70%)
* Rash (70%)
148
when are the most sx of HIV present?
in 1-4 weeks post exposure, after that they can get in a latent period and have less symtpoms
149
DDX of acute HIV
* Influenza
* EBV mononucleosis/ Primary CMV infx
* Severe (streptococcal) pharyngitis
* Secondary syphilis • Toxoplasmosis
* Drug reaction
* Viral hepatitis
* Primary HSV infection • Rubella
* Brucellosis
* Malaria
* West Nile Virus
