Heme Flashcards
1
Q
5 causes of decreased sed rate
A
Polycythemia
Sickle cell anemia
CHF
Microcytosis
Hypofibringonemia
1
Q
t(15:17)
A
PML (M3)
2
Q
What binds LPS on gram - cell membrane?
A
CD14 receptor of macrophages
2
Q
CD10+, TdT+
A
ALL
2
Q
Auer rod
A
AML
2
Q
Treatment of CML
A
Imatinib
(remember bcr-abl has tyrosine kinase activity)
4
Q
Baby with failure to thrive, megaloblastic anemia not corrected by B12 or folate
A
Orotic aciduria
4
Q
Mechanism of lymphopenia with GC use
A
Apoptosis
4
Q
Neuropathy in adults and encephalopathy in kids
A
Lead poisoning
5
Q
Defect in TTP
A
ADAMST13 = vWf MMP = long vWf = increased thrombosis and decreased platelet count
5
Q
Macrophages present antigen via:
A
MHC class II
(to CD4+, think granulomas)
5
Q
Cause of AIP
A
Defective porphobilinogen deaminase
6
Q
Universal donor of blood
A
O-
(no antigens on RBCs)
6
Q
What does hepcidin do?
A
Released from liver during inflammation, causing decreased iron release from intestine and macrophages –> ACD
7
Q
Alpha granules of platelets
A
Fibrinogen, vWf
8
Q
Cross-links fibrin
A
XIII
8
Q
Orotic aciduria vs. OTC deficiency
A
Orotic aciduria - defective pyrimidine synthesis due to UMP synthase deficiency = high orotic acid, normal ammonia, megaloblastic anemia
OTC deficiency - defective urea cycle, high orotic acid, high ammonia, no megaloblastic anemia
9
Q
Treatment of vW disease
A
DDAVP = increases stored vWf in endothelium
10
Q
Indirect Coombs
A
Add normal RBCs to patients serum –> add anti-IgG –> agglutinate if patient’s serum contained IgG against RBCs (AHA)
11
Q
Activates macrophages
A
IF-gamma
11
Q
Cryoprecipitate contains:
A
vWf, factors VIII and XIII, fibrinogen
12
Q
Which coagulation factor is carried by vWf?
A
VIII
13
Q
Increased risk of parvo B19 crisis
A
SCD, beta thal major
13
Q
A
PML (M3 AML)
(notice the fuck ton of auer rods)
14
Bite cell = G6PD deficiency
15
Sickle cell anemia
15
Precipitates AIP
Alcohol, starvation, phenytoin, griseofulvin, phenobarbital
15
Langerhans cell histiocytosis - lytic bone lesions in a kid
(S100+)
16
What do you give to increase clotting factors?
FFP
17
Two causes?
Hereditary spherocytosis
Autoimmune hemolysis
18
What is the "stippling" seen in lead poisoning and thalassemia?
rRNA (decreased degradation)
19
Treatment of PML?
ATRA - induces differentiation of myeloblasts
20
When does beta thal major manifest? Why?
6 months after birth - lose fetal Hb
21
Labs in ACD?
Decreased iron and TIBC, increased ferritin
21
Translocation of CML
bcr-abl, t(9:22)
22
Blood or tissue:
Monocyte, macrophage
Monocyte - macrophage precursor in blood
Macrophage - tissues
22
List the leukocytes from most to least abundant in normal blood
Neutrophil
Lymphocyte
Monocyte
Eosinophil
Basophil
22
bcl-2
Follicular lymphoma, anti-apoptotic oncogene
23
Medicinal cause of B12 deficiency
PPI's
23
Diagnosis of vW disease
No agglutination of ristocetin cofactor assay
24
Universal recipient of blood
AB
| (no antibodies)
25
Two pathways involved in subacute combined degeneration
Lateral CS tract
Dorsal columns
(Spasticity + defecits in vibration/proprioception; due to B12 deficiency)
26
Symptoms of AIP
Painful abdomen
Port wine-colored urine
Polyneuropathy
Psych disturbances
27
Beta thal major - marrow expansion
29
80% of circulating lymphocytes are:
T cells
30
Mast cells contain:
Heparin, eosinophil chemotactic factor, histamine
30
What causes Heinz bodies?
Oxidation of Hb sulfhydryl groups --\> denatured Hb precipitates
30
CML vs. leukomoid reaction
Both have left shift
CML has low ALP, leukomoid reaction has high ALP
31
Hyperammonemia + orotic acidemia
OTC transcarbamylase deficiency - no urea cycle so extra ornithine converted into orotic acid
(X-linked urea cycle deficiency)
31
CD15+, CD30+
RS cell, Hodgkin's lymphoma
32
Steps in primary hemostasis
Endothelial injury --\> exposed collagen ---\> vWf binds collagen --\> platelets binds vWf on endothelium via GpIb --\> platelets release ADP --\> better adherence to endothelium + upregulation of GpIIb/IIIa --\> platelets bind fibrinogen
32
RBC pathology seen in megaloblastic anemia
Macro-ovalocyte
32
Treatment for sideroblastic anemia?
B6 = cofactor for ALA synthase
32
S100+
Langerhans cell histiocytosis
Melanoma
Schwannoma
33
What causes Howell-Jolly bodies?
Nuclear remnants in RBC that cannot be cleared by macrophages due to asplenia/hyposplenia
34
Dense granules of platelets
ATP, calcium
35
4 infections that can cause aplastic anemia
Parvo
HIV
HVC
EBV
36
Most common cause of death in SCD adults
Acute chest syndrome
(vaso-occlusive disease of pulmonary vasculature)
37
Cyclin D1
Mantle cell lymphoma
38
Pseudo-Pelger-Huetz anomaly
(bilobed PMN after chemo)
39
t(8:14)
Burkitt lymphoma
39
TdT+
ALL
(marker of pre-B and pre-T cell)
40
Converts plasminogen to plasmin
Kallikrein and tPA
41
Treat with glucose
AIP (inhibits ALA synthase)
42
Diffuse large B-cell lymphoma translocation
t(14:18)
43
What is a "blast crisis"?
Transformation of CML into AML or ALL
44
JAK2 mutation
Polycthemia vera
Essential thrombocytosis
Myelofibrosis
(myeloproliferative D/O's other than CML)
45
Heinz bodies = G6PD deficiency
46
Acanthocyte
Liver disease, abetalipoproteinemia
46
How does adult T cell lymphoma present?
Lytic bone lesions and hypercalcemia
47
Lytic bones lesions and skin rash in a kid
Langerhands cell histiocytosis
48
CD14+
Macrophage
48
Mantle cell lymphoma marker
CD5+
49
Isolated basophilia
CML
51
Azurophilic granules of PMNs are actually:
Lysosomes
52
Causes?
HbC
Asplenia
Liver disease
Thalassemia
52
CLL
(CD5+, CD20+)
53
Heinz bodies = G6PD deficiency
54
HbC disease
Glutamic acid --\> lysine at position 6 of beta gene
Target cell
56
GpIb
vWf receptor
57
Co-stimulatory signal for T cell activation
CD28
57
Very low leukocyte alk phos
CML (vs. leukomoid reaction)
58
Defect in ITP
Autoantibodies against GpIIb/IIIa
60
Hereditary elliptocytosis
61
Cause of orotic aciduria?
UMP synthase deficiency = can't convert orotic acid to UMP in pydrimidine synthesis
63
Markers of ALL
TdT+, CD10+
64
Heparin, histamine, leukotrienes
Basophils
65
Increased HbA2
Beta thal minor
66
What causes the appearance of sideroblastics?
Too much iron in mitochondria
67
Hairy cell leumia, TRAP stain
69
Decreased haptoglobin
IV hemolysis
(Binds up all the free Hb and takes it to the RES, so reduced numbers in blood)
70
Causes of warm agglutinin AHA
Alpha-methyldopa
CLL
SLE
72
Defect in Bernard-Solier
GpIb
73
Howell Jolly bodies (asplenia)
74
ALL
(TdT+, CD10+)
74
How does Langerhans cell histiocytosis present?
Kid with rash + lytic bone lesions or recurrent otitis media with mastoid mass
76
Direct Combs
Add anti-IgG to patient's blood --\> agglutinate if IgG attached to RBCs (AHA)
76
Manifestation of essential thrombocytosis
Bleeding, thrombosis (increased number of ABNORMAL platelets)
78
Small, more numerous granules in PMNs contain:
Lactoferrin, lysozyme, alk phos, collagenase
80
Cell of type I hypersensitivity
Mast cell
81
How does CML differ from other myeloproliferative disorders?
No JAK2 mutation
+ t(9:22)
82
CD21+
B cell, binds C3d
82
Dry tap on BM aspiration
Myelofibrosis
Hairy cell leukemia
83
Follicular lymphoma translocation
t(14:18), bcl-2
84
Normal methymalonic acid but increased homocystine
Folate deficiency
85
Asplenia
(notice Howell Jolly bodies + target cell)
86
Problem in Factor V leiden?
Factor V is resistant to degradation by protein C = hypercoagulated
87
Markers of mature B cell
CD19, CD20, CD21
88
Normal platelet count and PT/PTT but prolonged bleeding time
Glanzmann thromboasthenia (no GpIIb/IIIa)
89
Asymptomatic precursor to MM
MGUS
90
Large, less numerous, azurophilic PMN granules contain:
beta-glucuronidase, MPO, acid phosphatase, proteinase
91
Leukemia associated with AHA
CLL
(warm or cold agglutinins)
92
CD5+
Mantle cell lymphoma (t11:14)
CLL
93
FoxP3
Treg
95
Defective ALA synthase vs. dehydratase
Synthase = sideroblastic anemia
Dehydratase = lead poisoning (+ defective ferrochelatase)
95
Two infections that cause hemolysis
Malaria
Babesia
96
M spike = mostly IgG with some IgA
MM
97
Stores fibrinogen
Alpha granule of platelet
98
Converts HMWK --\> Bradykinin
Kallikrein
99
Anemia + neuro symptoms
Lead poisoning
B12 deficiency
TTP
100
Who gets Mantle cell lymphoma?
Older males
101
HbH
ß4 - seen in three allele deletion in alpha thal
102
Langerhans cell histiocytosis (Birbeck granules)
103
CD20+
B cell
(think CLL)
105
Hemolytic anemia in a newborn
Pyruvate kinase deficiency
(No glycolysis --\> RBCs lyse)
106
One symptom shared by SCD and sickle cell trait
Renal papillary necrosis
(O2 tension in renal papilla low enough to induce sickling in SC trait)
108
vW disease vs. hemophilia A or B
vW disease will prolong bleeding time, while hemophilia is purely a clotting factor problem (increased PTT)
109
Prolong PTT and bleeding time with normal platelet count and PT
vW disease
110
Basophi granules
Heparin, histamine, leukotrienes
112
Oncogene of Burkitt lymphoma
c-myc (transcription factor)
114
RBC, WBC, platelet:
PV
Essential thrombocytosis
Myelofibrosis
CML
PV - everything increased
Essential thrombocytosis - just platelets increased
Myelofibrosis - RBCs decreased, others variable
CML - decreased RBCs, increased WBC and platelets
115
Better prognosis of ALL
t(12:21)
116
Two RBC pathologies seen in lead poisoning
Basophilic stippling + ringed sideroblasts
118
t(9:22)
Philadelphia chromosome - CML - BCR-ABL (tyrosine kinase)
119
G6PD deficiency cells
Bite cells + Heinz bodies
120
Predisposing factors include prior chemo, radiation, myeloprolierative disorder
AML
121
Follicular lymphoma oncogen
bcl-2
122
Protein C and S inactivate:
V and VIII
122
What is the function of haptoglobin?
Binds free hemoglobin and takes it to the spleen
123
During primary hemostasis, ADP is release from:
Platelet alpha granules to upregulate expression on GpIIb/IIIa
123
Pregnancy and OCPs increase production of (regarding iron):
Transferrin, so increased TIBC
125
Beta thal major or SCD - marrow expansion
126
+ Coombs means:
Autoimmune (must have had IgG on RBCs)
127
Origin of hairy cell leukemia
B cell
128
Why do neonates get IM vitamin K when born?
Lack enteric bacteria which produce vitamin K
130
Two jobs of kallikrein
1. HMWK --\> BK
2. Plasminogen --\> plasmin
132
6 drugs that can cause aplastic anemia
PTU
Methimazole
NSAIDs
Chloramphenicol
Benzene
Carbamazepine
133
CD3+
Mature T cell
(part of T cell receptor complex)
135
ALL translocation
t(12:21), associated with better prognosis
136
Where does vWf come from?
Platelets and endothelial cells
137
Peroxidase + cytoplasmic inclusion
Auer rod = AML
139
No reticulocytes despite anemia
Aplastic anemia
140
Normal PT/PTT but no increase in PTT after heparin
ATIII deficiency
(congenital, nephrotic syndrome, renal failure)
141
bcr-abl
CML (tyrosine kinase)
142
Treatment of beta thal
Blood transfusions
143
Two RBC pathologies in liver disease?
Acanthocytes
Target cells
144
Markers of Hodgkin's lymphoma
CD15+, CD30+
145
Proteinases, acid phosphatase, beta-glucuronidase, MPO
Azurophilic (lysosome) granules of PMN
146
Labs?
Increased iron and ferritin, normal TIBC
(Ringed sideroblasts - too much iron in mitochondria)
147
Anemia + bone deformations + hepatsplenomegaly
Beta thalassemia
(Bone malformations and hepatosplenomegaly due to expanded marrow and extramedullary hematopoeisis, respectively)
149
Painless waxing and waning lymphadenopathy
Follicular lymphoma
150
GpIIb/IIIa
Binds fibrinogen
151
Origin of RS cell
B cell (CD15+ and CD30+)
152
CD19+
B cell
153
Myelofibrosis
(tear drop cells)
155
Treatment for hereditary spherocytosis
Splenectomy
(macrophages in spleen keep removing defective RBCs)
157
Converts fibringogen to fibrin
Thrombin
158
Myeloproliferative disorders can transform into:
AML
160
B thal minor
Underproduced beta chain
Generally asymptomatic
HbA2 \> 3.5%
161
Sideroblastic anemia - too much iron in RBC
162
Increased methylmalonic acid + homocystine
B12 deficiency
163
Labs?
Decreased iron and ferritin, increased TIBC
164
Mantle cell lymphoma translocation
t(11:14); cyclin D1 to Ig
165
Drugs that can precipitate oxidant stress in G6PD deficiency
Sulfas
Primaquine
Dapsone
INH
Aspirin
Ibuprofen
Nitrofurantoin
167
Why does orotic aciduria cause macrocytic anemia?
Defective pyrimidine synthesis = no DNA = megaloblastic RBCs
169
3 forms of alpha thalassemia
1. Hb Barts - no alpha allele, gamma4, hyrdops fetalis
2. HbH - 3 defective alpha alleles, ß4
3. 1-2 alpha allele deletions, clinically insignificant
170
Enlarged megakaryocytes
Essential thrombocytosis, ITP
171
CD4+ skin lesions
Mycosis fungoides
172
Causes of sideroblastic anemia
ALA synthase deficiency
Copper deficiency
Lead poisoning
Alcohol
B6 deficiency
INH
Myelodysplastic syndromes
173
Least abundant leukocyte in normal blood
Basophil
174
Where in the anemia classification scheme do thalassemias fall?
Microcytic
174
What do you give to hemophilia patient?
Recombinant factor VIII or cryoprecipitate
175
Causes of cold agglutinin AHA
Mycoplasma
Mono
CLL
176
Protected against P. falciparum
Sickle cell TRAIT
177
MC non-Hodgkin lymphoma in adults
Diffuse large B-cell lymphoma (t14:18)
178
c-myc
Burkitt lymphoma (transcription factor)
179
Smudge cells
CLL
180
Treat with heme
AIP (inhibits ALA synthase)
181
Isolated basophilia
CML!!!!!!!!
183
How do you diagnose monoclonal gammopathy of undertermined significance?
M spike with \< 3 g/dl serum monoclonal protein
184
Bet thal major
Absent beta chain
Extramedullary hematopoeisis (hepatosplenomegaly)
Skull deformations (medullary expansion)
186
Mechanism of eosinopenia with GC use
Sequesters eosinophils in lymph nodes
187
Decreased platelet count + increased bleeding time
Bernard-Soulier (no GpIb)
ITP (Ab against GpIIb/IIIa)
TTP (no ADAMTS13 = vWf MMP)
189
Decreased platelet count + prolonged BT and PT/PTT
DIC
190
Wrist and foot drop in kid
Lead poisoning
(give SUCCIMER)
191
Infectious cause of megaloblastic anemia?
Diphyllobothrium latum (fish tapeworm) = B12 deficiency
192
Leukemia most associated with DIC
Promyelocytic leukmia (AML M3)
(can be induced by chemo --\> due to release of Auer rods)
194
Recurrent otitis media in kid (if cancer)
Langerhans cell histiocytosis
(ineffective Langerhans cells can't present to T cells)
195
Burkitt lymphoma translocation
t(8:14)
196
Heparin, histamine, eosinophil chemotactic factor
Mast cells
198
CD28+
Co-stimulatory signal for T cell activation
199
Eosinophils contain:
Arylsulfatase, heparinase
200
Hemolytic anemia + venous thrombis + pancytopenia
PNH (no GPI anchor for DAF)
201
t(11:14)
Mantle cell lymphoma; cyclin D1 to Ig
202
How do nephrotic syndrome and renal failure cause hypercoagulability?
Loss of ATIII in urine = increased factors II and X
203
Diagnosis of PNH
CD55/59 - on flow cytometry
205
Treatment of hairy cell leukemia
Cladribine (2-CA)
Adenosine analog resistant to ADA
206
3 functions of BK
1. Vasodilation
2. Pain
3. Increase permeability
207
CD10+
ALL
(Pre-B cell marker)
208
Labs in hemochromatosis
Increased iron and ferritin and % transferrin sat
Decreased transferrin/TIBC
209
Point mutation in SCD
Glutamic acid --\> valine at position 6 of beta gene
210
Two RBC pathologies in thalassemia?
Basophilic stippling
Target cells
211
Causes?
DIC
TTP/HUS
Prosthetic valve
212
Marker of macrophage
CD14 (binds LPS)
213
Skin necrosis after warfarin administration
Protein C deficiency = can't break down V or VIII and warfarin only breaks down II, VII, IX, and X
215
What substrate is present in the urine of an AIP patient during an attack?
Coporphobilinogen
216
Best prognosis for Hodgkin's?
Lymphocyte rich form
217
RBC pathology in abetalipoproteinemia
Acanthryocyte (spiny cell)
218
CD25+
Treg
219
Alk phos, collagenase, lactoferrin, lysozyme
PMN small, numerous granules
220
Triad of PNH
Hemolytic anemia + venous thrombus + pancytopenia
221
Defect in Glanzmann thromboasthenia
GpIIb/IIIa receptor
222
DIC vs. TTP
DIC = low platelet count + prolonged BT and PT/PTT
TTP = only platelets are jacked up, so PT/PTT are normal while platelet count is low and BT is prolonged
223
Pseudo-Pelger-Huet anomaly
Bilobed neutrophils (hyposegmented) typically following chemo
224
Two jobs of plasmin
1. Degrades fibrin
2. Activates complement (C3 --\> C3a)
225
What causes bite cells?
Removal of precipitated Hb by phagocytes
226
Who gets adult T cell lymphoma?
IVDA (HTLV-1)
227
Urobilinogen vs. unconjugated bilirubin in urin
Urobilinogen = IV hemolysis
Unconjugated bilirubin = EV hemolysis (also jaundiced)
229
Mycosis fungoides marker
CD4+
230
1-2 alpha chain allele deletions
No clincally significant anemia
231
M spike = IgM
Waldenstrom's macroglobulinemia (hyperviscosity syndrome)
232
Treatment of AIP
Glucose and heme, which inhibit ALA synthase
233
t(14:18)
Diffuse large B-cell lymphoma
Follicular lymphoma, bcl-2
234
Unique to Tregs
FoxP3, CD25+
235
Hb barts
Gamma4 - seen in 4 allele deletion of alpha thal - incompatible with life (hydrops fetalis)
