109 Anemia And Polycythemia Flashcards

1
Q

Anemia defined

A

Absolute decrease in circulating RBC

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2
Q

RBC structure

A

quaternary structure w/ 4 heme polypeptide subunits
bound to an iron molecule that is contained in porphyrin rin

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3
Q

RBC transport of o2 depends on what 3 things?

A

Hemoglobin
Affinity of oxygen
Flow of blood

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4
Q

What does a left shift of the oxy hemoglobin cube indicate?

A

Higher binding affinity of o2 and poor offload of tissues

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5
Q

What factors may cause a left shift of the oxy hemoglobin curve to the left (ie high o2 affinity and poor offload at the tissues?)

A

decrease temp
decrease 2-3 dpo (genetic sometimes)
decrease H ions (ie more basic)
CO
methemoglobin
fetal hbg

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6
Q

What factors may cause a RIGHT shift of the oxy hemoglobin curve to the left (ie low o2 affinity and greater offload at the tissues?)

A

incr temp
incr 2-3 dpo
incr H –> acidosis

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7
Q

What hormone does anemia stimulate and what does this do?

A

epo regulates production of rbc and differentiation of erythroid cells to this

stimulated by tissue hypoxia and rbc destruction products

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8
Q

What 4 main cells does the BM stem cells make?

A

erythroid
myeloid
megakaryocytic
lymphoid

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9
Q

Erythroid cells in BM make ?

A

rbc

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10
Q

Myleoid cells in BM make ?

A

granulocyte - baso, neutro, eosinophil and monocytes which in turn go into macrophage

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11
Q

Megakaryocyte cells in BM make ?

A

plt

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12
Q

Lymphoid cells in BM make ?

A

t and b cells from small lymphocytes, as well as NK cells

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13
Q

How long do RBC last in circulation?

A

110-120

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14
Q

Reticulocytes: released every ? d, MCV of __
normal range? (%)

A

1-3d
mcv 60
1-3%

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15
Q

DDX of MCV <81 (microcytic anemia)

A

TAILS
thal, anemia ckd, id anemia, lead intox, sideroblastic anemia (ie aud or heavy metals poison)

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16
Q

DDX MCV 81-100 (normocytic anemia)

A

ABCD: ac bl loss, bm fail, chr dis, destruction (hemolysis)
acute blood loss
ckd
chronic disease overall
hypothyroidism
bm suppression
hemolysis
g6pd
aplastic anemia

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17
Q

DDX of macrocytic anemia

A

FAT-RBC (fetal hb, alc, thyrd, reticulocytosis-sev hemolysis, b12, cirr)
b12 defic
folate defic
liver disease
reticulocytosis
myelodyspl syndromes
etoh abuse
drugs: hydroxyurea, AZT, chemo agents

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18
Q

What will the findings be for iron, TIBC and ferritin in microytic anemia, with iron deficiency cause?

A

decr iron
incr tibc
decr ferritin

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19
Q

What findings of ldh and haptoglobin will hemolysis and g6pd present with?

A

inc ldh
decr haptoglobin

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20
Q

If a coombs test is +, what kind of hemolytic anemia?

A

autoimm

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21
Q

If a peripheral smear has large oval shaped rbc, hypersegmented neutrophils, think…

A

b12 or folat defic

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22
Q

If a peripheral smear has schisocytes, helmet cells spherocytes or bite cells, think…

A

hemolysis
g6pd

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23
Q

If a peripheral smear has microcytic or hypochromic rbc, think…

A

IDAnemia
thal
sideroblastic anemia
chronic/late disease

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24
Q

What is sideroblastic anemia?

A

inherited x linked or acquired (isonaizid, chronic etoh, lead poison) where iron accumulates in mitochondria or erythryocyte precursors and heme synthesis is impaired

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25
Acute blood loss clinical features?
tachy hypot orthostasis light headed dyspnea pallor tachypnea thirst, ams, dec u/o
26
Hx and PE for clinically severe anemia
hx: out of hosp status/tx bleeding diathesis underlying disease prior transfusion current meds trauma y/no vitals: tachy, hypot, LOC, skin, cv abdo Skin: petec, ecchymosis, GI, GU
27
Symptoms of nonacute anemia
ch pain, decr ex tolerance, sob, weak, fatigue, dizzy, syncope ask re diet, drugs/fhx bl diathesis possibilities sites of blood loss at eye, resp, gi, gu, skin jaundice? cardipulmonary, LN
28
Diagnostic testing for anemia - standard
cbc with diff, retic count and periph smear, rbc indices type and s vs crossmatch aPTT INR ptt lytes glucose cr urinalysis for free hemoglobin
29
Diagnostic testing for anemia - fancy
folate, b12, tsh, retics, dat/coombs ldh/haptoglobin
30
When to consider adm for a nonurgent anemia:
1. cardiac or neuro sx 2. unexplained hbg or hct 3. difficulty with f/u
31
Management of iron deficiency anemia
Ferrous sulfate 325mg PO (65mg elem iron) TID in adults or 2mg/kg/day elem kids can see some improvement in 24 hours - retics 3-4d child, 1 week adult - full 4-6mo
32
Thalassemia: what is a normal HbA made up of ?
2 alpha paired globin chains 2 beta paired chains
33
Thalassemia: what is a normal Hbfetal made up of ?
2 alpha 2 gamma
34
Thalassemia: what is this?
AR - synthesis of abn globin chains in various combinations which can cause a hemolytic anemia as they apoptos, decr synthesis and ineff erythpropoeisis
35
Beta thalassemia what is this?
reduced/absent beta globulin synthesis w/ excess alpha globins can be silent ie carrier, minor or major
36
Tx major beta thal?
transfusion or chelation dependent
37
Alpha thalassemia: what is this?
reduced/abs alpha globin w/ excess of beta globulins Hemoglobin H is B globin tetramer vs Hemoglobin Bart
38
What are the transfusion dep thalassemias?
thal major severe hbg E/beta thal, Hbg barts hydrops need to survive
39
What are the non transfusion dep thalassemias?
thal minor mild hbgE HbH, alpha thal trait range no transf to intermittent
40
Clinical features of b chain thalassemia - when to consider?
Mediterranian pop - sev anemia, hepatosplenomegaly, jaundice, abn development and prem death usually by 2y transfusion dep - risk of iron OD in tissue so require chelator
41
Clinical features of heterozyg beta thalassemia/thal minor- when to consider?
some functional beta chains so a mild anemia, usually asx with no transfusion
42
Clinical features of alpha chain thalassemia - when to consider given how many alpha thal are abnormal (1-4)
asx carrier - prenatal death silent carrier - one missing gene alpha thal trait 2- missing genese Hemoglobin H - 3 missing mild mod anemia Hb barts all 4 abn alpha genese = hydrops fetalis and fetal death
43
Thalasemia testing
microcytic anemia hypochromic on peripheral smear with target cells and basophilic stippling needs electrophoresis and genetic testing
44
Silent carrier, beta thal minor and alpha trait thal - tx?
none unless times of high stress like infection, pregnancy, surgery, low hbg
45
Treatment of transfusion dep thalssemias?
transfusion, for goals of correcting anemia, suppressing ineff epo, inhibit incr gi abrorp q2-5 weeks to aim for 90-105
46
Transfusion dependent thalssemias: what to do in terms of iron chelators?
deferoxamine, deferiprone, deferasirox
47
Why give hydroxyurea in some Non transfusion dependent thals?
cytotoxic antimetabolite incr the fetal hemoglobin
48
Non and Transfusion dependent thalssemias: type of surgery vs longer term/more invasive management strategies?
splenectomy hematopoeitc stem cell transplant
49
Sideroblastic anemia: defect in ? synthesis
porphyrin
50
Sideroblastic anemia: idiopathic - what to worry about in elderly?
common cause refractory anemia elderly and may be pre leukemic state
51
Sideroblastic anemia: acquired examples
chloramphenicol, isonaizid, linezolid, penicillamine myelodyspl syndrome or myeloprolif disorders etoh copper defic, lead poison, zinc tox
52
Sideroblastic anemia: pathophysiology
impairs ability to make hbg and the excess iron ends up in mitochondria
53
Sideroblastic anemia: levels of serum iron, ferritin, tsat?
all high
54
Sideroblastic anemia: characteristic smear?
ringer sideroblasts - at least 5 on prussian blue stain which is ++ erythroid precursor
55
Sideroblastic anemia: management of congenital/acquired (as most times tx is tx underlying)
pyridoxine (B6) and responds to tx with 100mg PO TID sometimes ongoing transfusion and chelation soemtimes st transplant if congenital or myelodysplastic
56
Anemia of CKD: what happens to RBC and erythropoesis?
reduced making and survival time as hostile inflamm environment
57
Anemia of CKD: 5 causes
malignancy arthritis renal insuff HF copd chronic infection
58
Anemia of CKD: diagnostic testing and lab values of serum iron, tibc, ferritin and BM
cbc mcv low serum iron low TIBC normal/elevated ferritin normal BM
59
Megaloblastic anemia 2 main causes
folate f12
60
Non megaloblastic causes - name 5
etoh liver dysfunc hypothyroid myelodysplastic syndromes drug: hydroxyurea, methotrexate, zidovudine, vpa
61
Vi B12 defic clinical findings
paresthesia hand and feet, decreased proprioception, vibratory sense, weakness/spasticity of LE can be forgetful, depressed, paranoid degen of SC due to loss myelin in dorsal and lateral column - movement related *ataxia
62
What aspect of vit b12 deficiency does folate deficiency present with?
neuropsych
63
Diagnostic testing of a megaloblastic anemia
mcv >100 peripheral smear: large oval red cell, anisocytosis hypersegm PMN elevated LDH b12 and folate levels
64
How to treat folate defic
1mg oral folic acid/day
65
How to treat B12 deficiency?
check out causes of malabsorption Parenteral 1000mcg IM daily 7-10d then q1mo
66
Aplastic anemia causes - non infectious, name 5
chloramphenicol phenylbutazone anticovulsants insecticides solvents sulfoonamides gold benzene
67
Aplastic anemia name 5 non drug causes
autoimm viral hepatits radiation exposure viral illness - parvo b12, hiv, ebv
68
What med to avoid in aplastic anemia?
aspirin
69
What is a myelodysplastic syndrome?
class hematopoetic stem cell disorder characterized by cytopenia, myelodysplasia, ineffective hematopoesis that oncogenic mutations cause
70
myelodysplastic syndrome on BM bx - findings?
dysplastic cell lines blast cells
71
Myeloproliferative sydromes - what is primary myelofibrosis?
primary BM fibrosis, splenomegaly due to extramedullary hematopoeisis --> can transform to leukemia
72
Clinical features of hemolytic anemia in general?
sx of anemia new jaundice, hematuria, fever, hepatosplenomegaly, abdo/back pain, AMS
73
Hemolytic anemia: make sure you ask about ...
fam hx pmhx
74
How does intravascular hemolysis pathophys work?
decr o2 carrying capacity, release of free hemoglobin that binds to haptoglobin and hemopexin, to liver, conjugated to bili and extcreted = jaundice this system gets overwhelmed = hemoglobinuria, hemoglobinemia
75
What are some general causes of intravascular hemolysis?
abo incompatibility autoimmune hemolytic anemias infection DIC toxins
76
What are some causes of extravascular hemolysis?
hereditary spherocytosis g6pd scd autoimmune
77
Name 5 causes of intrinsic hemolytic anemia
PK defic, g6pd spherocytosis, PNH hemoglobinopathies, thal
78
Name 5 causes of extrinsic hemolytic anemia
alloantibodies/auto microangiopathic hemolytic anemia, prosth heart valve drugs toxins infections thermal abnormal sequestration
79
G6PD - how does this cause intravascular hemolysis?
glutathion needed to protect hemoglobin from oxidant injury - some AA or medit decent to not have this
80
Drugs causing incr hemolysis in G6PD - name 5
aspirin primaquine quinine nitrofurantoin sulfamethoxazole sulfones fava beans methylene blue nalidixic acid
81
Hereditary spherocytosis: what is this?
AD mutation in gene so the RBC membrane is shaped as a sphere and thus easily friable by spleen
82
Hereditary spherocytosis: how to tx?
supportive they probably RQ splenectomy >6y if severe
83
Hereditary spherocytosis: MCHC and RDW?
incr
84
Paroxysmal nocturnal hemoglobinemia (PNH): what is this?
stem cell defect causing abnormal erythrocyte, neutrophil and plt sn to complement
85
Paroxysmal nocturnal hemoglobinemia (PNH): name 4 causes
chronic hemolysis hemosiderinuria leukopenia low plt
86
Paroxysmal nocturnal hemoglobinemia (PNH): lab findings - smear, cooms, Ham test?
n, neg ham - normal activation with complement with use sucrose or acid hemolysis
87
Paroxysmal nocturnal hemoglobinemia (PNH): key complication?
thrombosisc of hepatic vein
88
Paroxysmal nocturnal hemoglobinemia (PNH): tx
support eculizumab
89
ABO incompatibility: how does this work?
IgM abodies act as hemolysin causing agglutination of RBC and fixation of complement so you get intravascular hemolysis
90
Rh incompatibility how does this work for hemolytic anemia?
IgG causes accelerated destruction of RBC by spleen/liver
91
Diseases assoc with autoimmune hemolytic anemias: name 4 categories
neoplasm collagen vascular disease infections misc - thyroid, uc, drug immune reactions
92
Diseases assoc with autoimmune hemolytic anemias: neoplasms
mlaign: CLL, lymphoma, myeloma, thymom, CML benign - teratoma, dermoid cyst
93
Diseases assoc with autoimmune hemolytic anemias: collagen vascular disease
sle periarteritis nodosa RA
94
Diseases assoc with autoimmune hemolytic anemias: infections
mycoplasma syphilis virus: mono, hepatitis, influenza, coxsackie, cmv
95
Autoimmune hemolytic anemia - warm type often seen in?
yo female
96
Autoimmune hemolytic anemia - warm - how does this occur?
variable complement fixation, + DAT for IgG as it binds to Rh, is removed by macrophages and sequested in spleen
97
Autoimmune hemolytic anemia - warm tx
supportive - manage underlying GC, rituximab second line is things heme gives IVIG< plex and surgery for splenectomy
98
Autoimmune hemolytic anemia - cold - who is this seen in?
old men
99
Autoimmune hemolytic anemia - cold - how does this occur?
IgM complement fixation which react with surface ag at low temps and cause either intra or extra vascular hemolysis upon rewarming
100
Autoimmune hemolytic anemia - cold - 3 common diseases?
ebb mycoplasma lymphoma
101
Autoimmune hemolytic anemia - cold - DAT ?
+
102
Autoimmune hemolytic anemia - cold - tx
supportive with folate and vit b12 if defic avoid trigger underlying disease tx
103
Autoimmune hemolytic anemia - cold - complication?
thrombosis - may consider thromboprophylaxis with acute exacerbations or for chronic disease in high risk situations
104
Extrinsic cause of hemolysis: how does microangiopathic hemolytic anemia occur?
microcirculatory fragments by threads of fibrin deposited in arterioles
105
microangiopathic hemolytic anemia - underlying disease- list 6
malign htn preeclampsia vasculitis ttp purpura dic vascular anomalites
106
Extrinsic cause of hemolysis: Cardiac - how does this occur?
shearing from turbulance - ie valves, av fistula, AS, LS heart lesion
107
Extrinsic cause of hemolysis: march hemoglobinemia
breaking IV rbc by repetitive pounding - soldiers and marathoners
108
What are environmental/toxin causes of hemolysis? List 7
burns freshwater drown hyperthermia brown recluse spider venomous snake bite castor beans mushrooms malaria bartonella clostridium
109
What is a DAT test for ?
hemolytic anemia - detects abodies or complement on human rbc membranes by adding a reagant that detects igG, A M or complement
110
How to temporize/slow hemolysis - drug?
prednisone 1mg/kg
111
What is sickle cell disease?
AR mutation producing HbS as a mutation in he beta globin gene
112
In sickle cell, what happens to rbc when exposed to deoxygenation or various stressors?
rbc sickles, rigid so that blood becomes sludge, undergoes sequestration in liver and spleen
113
Sickle cell disease: complications list 6
vaso occlusive events chronic hemolysis thrombosis organ injury - renal papillary necorsis, splenic infarct, PE, traumatic hyphema, exertional rhabdo, sudden cardiac death
114
Sickle cell disease: when to consider life threatening aplastic crisis?
hbg <20 after previously stable and retic count <2% - *can occur after suppression epoesis infection or folate defici
115
Sickle cell disease: Acute vaso occlusive event: precip factors?
antecedent infection cold exposure stress/trauma
116
Painful Sickle cell disease crisis - what happens?
origin in tissue ischemia which leads to irev sickling, more sludge and obstruction
117
Painful Sickle cell disease crisis which body parts typically involved?
abdo chest back extremities
118
Sickle cell disease neuro diseases?
tia cerebral infarct ich scinfarct vestibular hearing issue
119
What is an acute chest syndrome?
SCD: fever cough hypoxia, chest pain, sob and new infiltrate on cxr look for mycoplasma/chlam infection
120
When does autoinfarction of spleen occur in kids with sickle cell?
10-27mo
121
Functional asplenia in sickle cells kiddos: 1mo-5y how to tx?
amox/pen
122
What bugs are people with scd more at risk for getting?
encapsulated organisms salmonella om!!
123
Treating a sickle cell patient - important considerations:
1. hydroxyurea: induces HbgF, reduces pain crisis and need blood transfusion 2. transfusion for severe exacerbation with evidence of hypoxia/hypoperfusion, acute organ damage, preop 3. exchange transfusion if acute chest syndrome, stroke, asplastic crisis, acute splenic sequestration 4. stem cell transplant: cure!!
124
Acute pain crisis tx
analgesia nsaids ivf
125
Stroke in SCD pt:
exchange transfusion with goal hbs <30% and hbg of 10 tpa in adults also consideration
126
Acute chest syndrome management of scd:
hydration analgesia oxygenate/ventil CTX and azithromycin exchange transf if multilobe involvemnt or worsening hypoxemia, neuro abn, multi organ involvement
127
What GU concern is more common in SCD?
priapism - aspirate blood and irrigate with alpha adrenergic agent
128
What are the symptoms of polycythemia?
mild headache vertigo/dizzy, blurry vision due to vol oload hyperviscosity: thrombosis, plt dysfunc (epistaxis, spont bruising, GIB)
129
What are the signs of polycythemia?
plethoric skin/mm optic fundal venous congestions splenomegaly hf
130
Causes of absolute erythrocytosis: list 8
R to L shunt pulmonary disease carboxyhemoglobinemia high alt acclim sleep apnea high affnity hemoglobins FSGS, renal transplant hepatoma, adrenal tumor, meningioma, pheo, PV meds: steroids
131
Causes of absolute erythrocytosis: list 5
emesis, diarrhea, diuretics, burns, low albumin chronic plasma contraction: hypoxia, hypertension, tobacco or etoh use
132
What is primary polycythemia vera?
chronic myeloprolif disorder caused by JAK2mutation
133
polycythemia vera: symptoms
h/a weak dizzy excess sweat plethora pruritis after hot water exposure
134
polycythemia vera list 3 complications of disease
cva, mi, dvt bleeding risk leukemic or fibrotic transformation
135
Labs: polycythemia vera
all cell lines involved and typically big with elevated hbg, rbc mass, plt and wbc big
136
polycythemia vera diagnostic criteria
hemoglobin >16.5 in men, 16 in wo or hct >49% men, 48% wo or incr rbc mass bm trilineage prolif with pleom mature megakaryocytes jak2 mutation
137
polycythemia vera tx
1. phlem to hct <45% 2. daily aspirin 40-100mg po for primary prevention thrombosis 3. high risk (hx thrombosis, age >60: hydroxyurea 500mg po bid
138
Secondary polycythemia: defn?
appropr epo response to abn tissue oxygen levels (ie excluded by N art o2 sat)
139
Secondary polycythemia: emergent tx
phlebotomy - 500ml and replace with saline same true emerg = 1-1.5L of blood over 24 hour period