Hemo Flashcards
(153 cards)
0
Q
Bite cells
A
G6PD
1
Q
Heinz body
A
G6PD
Heinz body like inclusion seen in alpha-thalassemia
oxidation of iron from ferrous to ferric form leads to denatured hemoglobin precipitation and damage to RBC membrane–>formation of bite cell
2
Q
Russell bodies
A
Plasmacytoid that have stored immunoglobulin in the cytoplasm
3
Q
Rob like tubular birbeck granules
A
Langerhans cell proliferation
4
Q
Decrease in WBC especially in neutrophil
A
Overwhelming infection that cause increased peripheral use of neut at the site of inflammation
5
Q
RBC
A
life span:120d
source of energy is glucose
chloride-HCO3 antiporter
6
Q
Erythrocytosis
A
polycythemia
increase hematocrit
7
Q
Anisocytosis
A
RNC vary in size
8
Q
poikilocytosis
A
RBC varying shapes
9
Q
Reticulocyte
A
immature RBC, marker of erythroid proliferation
10
Q
PLT
A
derived from megakaryocyte
life span 8-10d
contains dense granules(ADP, Ca) and alpha granules (vWF,fibrinogen)
1/3 of PLT is in spleen
11
Q
vWF receptor
A
GpIb
12
Q
Fibrinogen receptor
A
GpIIb/IIIa
13
Q
Leukocytes
A
divided into granulocytes: neut, eosinophil, basophil
and mononuclear cells: monocytes, lymphmocyte
14
Q
Neutrophil
A
multilobed nucleus
small more numerous specific granules contain alkaline phosphatease, collagenase, lysozyme, and lactoferrin
Larger less numerous azurophilic granules(lysosomes),acid phosphatease, peroxidase and beta-glucuronidase
15
Q
Neut in Vit B12 deficiency?
A
Hypersegmented polys(>5)
16
Q
increase band cell reflects?
A
immature meut
myeloid proliferation
17
Q
Moncyte
A
Differentiate into macrophages in tissues.
Large kidney-shaped nucleus
extensive “frost glass” cytoplasm
18
Q
Macrophages
A
long life in tissue differentiate from monocyte in blood activated by gamma-IFN APC via MHCII CD14
19
Q
eosinophil
A
helminthic infection bilabate nucleus eosinophilic granules of uniform size high phagocytic for Ag-Ab complex produces histaminase and arysulfatase, help limit reaction
20
Q
eosinophil is cuzed by?
A
Neoplastic Asthma Allergic Collagen Parasite
21
Q
Basophil
A
mediates allergic reaction
densely basophilic granules
heparin, histamine, LTD4
22
Q
mast cell
A
allergic reaction
resembles baso
bind Fc portion of IgE to membrane
igE cross-links upon Ag binding–>degranulation–> histamine,heparin,eosino chemofactor
23
Q
DC
A
phagocytic APC
link between innate and adaptive immune system
Express MHCII and Fc receptor
Langerhans cell in the skin
24
T cell
mediates cellular immune response
25
Lymphocyte
round, densely stained large nucleus with small amount of pale cytoplasm
26
Plasma cell
off center nucleus
clock face chromatin distrubution
abundant RER
well developed Golgi apparatus: intensely stained
27
NK cell
large cytoplasmic granule
| kill tumor and viruse infected cell targets Ab coat and those without MHC
28
anti A and anti B are Ig?
| anti-Rh is Ig?
AB are IgM do not cross placenta
| Rh is Ig G, do cross placenta
29
Acanthocyte
| spur cell
liver disease
abetalipoproteinemia
state of chol dysregulation
30
Basophilic stippling
Thalassemia
Anemia of chronic disease
Lead poisoning
31
Elliptocyte
Hereditary elliptocytosis
32
Macro-ovalocyte
megaloblastic anemia, also hypersegmented PMNs
| marrow failure
33
ringed sideroblasts
sideroblastic anemia
| excess iron in mitochondria=pathologic
34
Schistocyte
| helmet cell
DiC
TTP/HUS
traumatic hemolysis
metal heart valve prosthesis
35
sickle cell
sickle cell anemia
36
spherocyte
hereditary spherocytosis
autoimmune hemolysis
with no central pallor
37
Teardrop cell
BM infiltration
myelofibrosis
骨髓纤维化：胶原再生，造血功能障碍
38
Target cell
```
HbC disease
Asplenia
Liver disease
Thalassemia
HALT! said the hunter to the target
```
39
Howell-Jolly bodies
Basophilic nuclear remnants found in RBCs
Howell-Jolly bodies are normally removed from RBCs splenic macrophages
Seen in patients with functional hyposplenia or asplenia, or after mothball ingestion (naphthalene)
40
Pappenheimer body
iron-containing
| dark-blue granules found in Wright-stained RBC in sideroblastic anemia
41
Iron is regulated by?
hepcidin, polypeptide hormone
| produced by liver
42
iron deficiency iron labs?
decrease iron
increase TIBC
decrease ferritin
43
koilonychia
spooning of nail in iron deficiency
44
transferrin
transported in plasma by iron-binding glycoprotein
synthesized in the liver
the mean function is to deliver Fe to cells, including RBC precursors
45
storage of Iron is through?
ferritin: liver (parenchymal cell), spleen(Macro), bone and skeleton muscle
hemosiderin: intracellular iron is located in cystol and in lysosome(turn blue-blac whem expose to potassium ferrocyanide->rpussian blue stain)
46
alpha thalassemia
alpha-globin gene mutation-->decrease alpha-globin synthesis
chrom 16
cis deletion, asian
trans: african
47
Hb Barts
excessive gamma-globin forms gamma4
in 4 gene deletion
incompatible with life
48
HbH disease
3 gene deletion of alpha thalassemia
| little alpha, excessive beta-globin forms beta4 (HbH)
49
beta thalassemia
point mutation in splice sites and promotor sequences
decrease beta-globin synthesis
Chron11
50
HbA2>3.5%
beta thalassemia minor
| beta chain is underproduced
51
crew cut on skull x-ray
marrow expansion-->skeletal deformities
beta-thalassemia, sickle cell anemia
Chipmunk facies
52
beta-thalassemia major
beta chain is absent-->severe anemia-->blood transfusion--> hemochromatosis
major-->increase HbF (alpha2gamma2)
53
HbS/beta thalassemia heterozygote
mild to moderate sickle cell disease depending on amount of beta-globin production
54
Lead poisoning
Lead line on gingivae(burton's lines) and on metaphyses of long bone
Encephalopathy, Erythrocyte basophilic stippling
Abdominal colic and sideroblastic Anemia
Drops-wrist and foot drop, Dimercaproll and EDTA are 1st line treatment
55
sideroblastic anemia
X-linked defect in delta-ALA synthase gene defect in heme synthesis
reversible etiologies: alcohol, lead and isoniazid
ringed sideroblasts, with iron-laden mitochondria
increase iron, normal TIBC, increase ferritin
treat with pyridoxine
56
Hypersegmented Neut
folate deficiency
| B12 deficiency
57
Non-Megaloblastic Anemia Causes
- Liver dz.
- Alcoholism (macrocytosis and bone marrow suppression can occur in the absence of folate B12 deficiency).
- Reticulocytosis. increase MVC
- Metabolic disorder (e.d., orotic aciduria): congenital deficincies of purine or pyrimidine synthesis.
- Drugs: 5-FU, AZT, hydroxyurea.
58
decrease haptoglobin
increase LDH
hemoglobin in urine
intravacular hemolysis
59
macrophage in spleen clears RBC
increase LCH and UCB
jaundice
Extracellular hemolysisq
60
Aplasic anemia
Pancytopenia.Normal morphology, but hypocellular bone marrow with fatty infiltration.
Causes: Failure or destruction of myeloid stem cells :
- Radiation and drugs (benzene, chloramphenicol, alkylating agents, antimetabolites).
- Viral agents (parvovirus B19, EBV, HIV, HCV).
- Fanconi's anemia (inherited defect in DNA repair).
- Idiopathic (immune mediated, primary stem cell defect); may follow acute hepatitis.
Tx: Withdrawal of offending agent, immunosuppressive regimens (antithymocyte globulin, cyclosporine), allogeneic BMT, RBC and platelet transfusion, G-CSF or GM-CSF.
61
anemia of chronic diease
inflammation-->increase hepcidin->inhibiting Fe transport-->decrease release of iron from macrophage
decrease iron, decrease TIBC, increase ferritin
62
chronic kidney disease
decrease erythropoietin
| decrease hematopoiesis
63
myeloplothisic anemia
replacement of BM by space occupying lesion
leukemmia/metastatic cancer
fibrosis-chronic idiopathic myelofibrous
64
pure RBC aplasia
| PRCA
inhibition of erythropoietic precursors and progenitors by IgG Ab/CTL
so it is associated by thyoma/ LL
check chest XR
65
Hereditary Spherocytosis
Extravascular hemolysis.Premature removal of RBCs by spleen.
Defect in ankyrin, band3, 4.2, or spectrin.
small round RBC without central pallor(Increased MCHC and RDW).
Findings: splenomegaly, aplastic crisisB19. Howell-Jolly post asplenia
Labs: Positive osmotic fragility test.
Tx: Splenectomy.
66
G6PD
Intravascular hemolysis. X-linked.
Defect in G6PD leads to decreased glutathione and increased RBC susceptibility to oxidant stress.
Findings: Hemolytic anemia following oxidant stress (e.g.sulfa drugs, infections, fava beans).
Sx: Back pain, hemoglobinuria a few days later.
Labs: Blood smear w/ RBCs with Heinz bodies and bite cells.
67
pyuvate kinase deficiency
Extravascular hemolysis. AR.
Defect in pyruvate kinase causing decrease ATP and rigid RBCs.
Na/K/ATP pump
Presentation: Hemolytic anemia in a newborn.
68
HbC defects
Extravascular
Glutamic acid-to-lysine mutation at position 6 in chain mutation.
Pts with HbSC (1 of each mutant gene) have milder dz than do HbSS patients.
69
Paroxysmal nocturnal hemoglobinuria
Intravascular. acquired mutation in a hematopioetic stem cell
Decay-accelerating factor (DAF)
Impaired synthesis of GPI anchor, DAF, and all GPI linked proteins in the RBC membrane that inhibits complement on RBC membrane.
Labs show increased CD55/59 - on RBC, RBC lysis on surcose/scid(sucrose lysis test and ham's test)
70
PNH triad
hemolytic anemia
pancytopenia
venous thrombosis
71
sickle cell anemia
-point mutation cause a single AA replacement of glutamate by valine
HbS position 6
-low O2/dehydration-->sickling(HbS polymerizes)-->anemia,occlusion
-Heterozygote resistance for malaria
72
new born are initially asymptomatic of sickle cell at first because?
increase HbF and decrease HbS
73
complication of sickle cell anemia
Aplastic crisis
Autosplenectomy,howell-jolly body-->increase risk for encapsulated organism.
Salmonella osteomyelitis
Painful crisis(vaso occlusion):dactylitis, painful hand swelling
Renal papillay necrosis and micro hematuria
74
warm agglutinin
IgG. warm weather goodddd
| Chronic anemia seen in SLE, CLL, or with certain drugs (e.g., alpha-methyldopa).
75
cold agglutinin
IgM., cold icecream yummmmm
Acute anemia triggered by cold.
Seen in CLL, Mycoplasma pneumoniae infections or infectious mononucleosis.
76
direct coomb
anti Ig antibody+patients serum(RBC,Ag)
77
indirect coomb
normal RBC+ patient serum(Ab)
78
microangiopathis anemia
RBC are damaged when passing through obstructed or narrowed vessel
DIC, TTP-HUS, SLE and malignant HTN
schistocyte are seen on blood smear due to mechanical damage to rbc
79
macroangiopathic anemia
prosthetic heart valves and aortic stenosis
| schistocyte
80
heme synthesis enzyme deficiency?
1.Ferrochelatase and ALA-hydratase--Lead poisoning
accumulation of delat-ALA, protoporphyrin
2.Porphobilinogen deaminase--acute intermittent porphyria
accumulation of porphobilinogen, delta-ALA,uroporphyrin
3.Uroporphyrinogen decarboxylase--porphyria cutanea tarda
accumulation of uroporphyrin
81
Acute Intermittent Porphyria
Sx: Painful abdomen, red wine-colored urine, polyneuropathy, psychological disturbances, precipitated by drugs.
Tx: Glucose and heme, which inhibit ALA synthase.
82
Porphyria Cutanea Tarda
most common prophyria
| Sx: Blistering cutaneous photosensitivity.
83
PT
extrinsic
84
PTT
intrinsic
85
hemophilia A
VIII, increase PTT
| hemarthroses, bleeding into joints, easy bruising, dental bleeding
86
hemophilia B
IX, increase PTT
| hemarthroses, bleeding into joints, easy bruising, dental bleeding
87
Vit K deficiency
increase PT and PTT
| 2,7,9,10, protein C and S
88
Bernard-Soulier syndrome
decrease PC
increase BT
defect in PLT plug formation
decrease Gpib-->defect in plT-vWF adhesion
89
Glanzmann's thrombasthenia
increase BT
normal PC
defect in PLT plug formation
decrease gpiib/IIIa-->defect in PLT-PLT aggregation
90
Idiopathic Thrombocytopenic Purpura (ITP)
```
PC: Decreased
BT: Increased
Mechanism: Decreased platelet survival.
Anti-GpIIb/IIIa antibodies cause peripheral platelet destruction.
Labs show increased megakaryocytes.
```
91
Thrombotic Thrombocytopenic Purpura (TTP)
PC: Decreased
BT: Increased
Decreased platelet survical.
Deficiency of ADAMTS 13 (vWF metalloprotease) causes decreased degradation of vWF multimers.
Pathogenesis: Increased large vWF multimers cause increased platelet aggregation and thrombosis.
Labs show schistocytes and increased LDH.
Sx: Pentad of neurologic and renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia.
92
TTP DD HUS
HUS have no CNS involvement
93
HUS
| hemolytic uremic syndrome
PC normal, BT increase
microangiopathic hemolytic anemia, acute renal failure&thrombocytopenia
shigella toxin
hemolytic anemia cause pallor, weakness and tachy
decrease Hb, increase LDH reticulocyte
94
von Willebrand's Disease
PC: No Change,BT: Increased
PT: No Change, PTT: Same or Increased
Intrinsic pathway coagulation defect: Decreased vWF (acts to carry/protect factor VIII).
Defect in platelet plug formation as decreased vWF leads to a defect in platelet-to-collagen adhesion.
Mild, but most common inherited bleeding disorder. AD.
Tx: DDAVP releases vWF stored in endothelium.
95
ristocetin cofactor assay
von willebrand
96
DIC
PC: Decreased
Increased BT, PT, PTT
Widespread activation of clotting leads to a deficiency in clotting factors.
Labs: Schistocytes, increased fibrin split products, decreased fibrinogen, decreased factors V and VIII.
97
Factor V Leiden
Production of mutant factor V that cannot be degraded by protein C.
most common hypercoagulation in white
98
Prothrombin Gene Mutation
Mutation in 3' untranslated region-->increase production of prothrombin-->venous clots.
99
ATIII Deficiency
Inherited deficiency of antithrombin.
| Reduced increase in PTT after administration of heparin.
100
Protein C or S Deficiency
Decreased ability to inactivate factors V and VIII.
| Increased risk of thrombotic skin necrosis with hemorrhage following administration of Warfarin.
101
blood transfusion risk?
```
infection
transfusion reaction
iron overload
hypocalcemia, citrate is a Ca chelator
hyperK, RBC may lyse in old blood units
```
102
Hodgkins and non-hodgkins LN involvement
single group in H, multiple in non H
extranodal is rare in H, common in NH
contiguous spread in H, non con in NH
stage is strongest predictor
103
NH lymphoma involves in what cell?
B-cell major
104
NH age group?H age group
20-40 for NH
| H is old or very young
105
Hodgkins is associated with what virus?
EBV
106
RS cell?
CD30+ and 15+,if lymphocyte dominant then it is the opposite 45+, CD 30 and 15 -
owl eyes
107
beter rognosis for Hodgkin's lymphoma happens in?
strong stromal or lymphocytic reaction against RS cells
| nodular sclerosing have the best prognosis in all and the most common type
108
Lacunar RS cell is found in?
large inclusion like nuclei and surround by halo
found in nodular sclerosis
background cell are mixed bands of fibrousis
common in young adult M=F
stage I/II
109
classic RS is found in?
nodular sclerosis
mixed cellularity
lymphocyte rich
lymphocyte depleted
110
mononuclear RS is found in?
mix cellularity
| lymphocyte rich
111
variant RS is found?
Lymphocyte depleted type with few diffuse fibrosis
| found in old and HIV very rare
112
L&H popcorn cell is found in?
nodular lymphocyte predominant type
back ground is B-cell and DC cell
uncommon happen in young male in cervical and axillary
113
Lymphocyte rich hodgkins is rich in?
T-lymphcyte
| found in older male
114
poor prognosis hodgkins
mixed cell
115
t(8,14)
burkitt's lymphoma
| B-cell lymphoma
116
translocation of c-myc and heavy chain ig(14)
burkitt's lymphoma
| B-cell lymphoma
117
EBV
burkitt's lymphoma
| B-cell lymphoma
118
"Starry sky" appearance
burkitt's lymphoma
sheets of lymphocytes with interspersed macrophages
B-cell lymphoma
119
t(14,18)
diffuse large B-cell lymphoma
B-cell lymphoma
most common adult NHL
120
bcl-6
| may be EBV and HHV-8
diffuse large B-cell lymphoma
| B-cell lymphoma
121
t(11,14)
mantle cell lymphoma, 4 l's
| B-cell lymphoma
122
translocation of cyclin D1 and heavy chain Ig
mantle cell lymphoma, 4 l's
poor prognosis CD5+
B-cell lymphoma
123
t(14,18) bcl-2
follicular lymphoma, 4"l",-->14, 2"o"-->18
| B-cell lymphoma
124
translocation of Ig heavy chain and bcl-2
follicular lymphoma
hard to cure
indolent course
bcl-2 inhibits apoptosis
125
Adult T-cell lymphoma
HTLV-1
| cutaneous lesion
126
mycosis fungoides
| sezary syndrome
adults present with cutaneous patches/nodules
CD4+
indolent course
127
fried egg appearance cancer
MM, monoclonal plasma cell cancer
128
large amount of Ig A and Ig G productionM spike on protein elctrophoresis
MM
129
Ig light chain in urine, bence johns protein
MM
130
Rouleaux formation
| RBC stacked like poker chips
MM
131
numerous plasma cell with clock face chromatin and intracytoplasmic inclusions containing Ig
MM
132
MM is associated with amyloidosis?
yes, primary A, AL
133
```
CRAB
hyper Ca
Renal insufficiency
Anemia
Bone lytic
```
MM
| punched out lytic bone lesion on x-ray
134
M spike but no lytic bone lesion
Waldenstrom's macroglobulinemia
| also have: visual impairment, neurological problem, bleeding and anemia
135
M spike with no symptoms
MGUS, precursor of MM
| monoclonal gammopathy of undetermined significance
136
plasmacytoma
can become MM
| surgical resection
137
leukemoid reaction vs CML
both have increase WBC with shift to the left
CML have a decrease leukocyte alka phosph
leukemoid have a increase alka phosph
138
mummification of RS cell
shrunken and pyknotic and not as large
| RS cells
139
ALL
better prognosis
| responsive to therapy,becareful for kidney stone in treatment: hydration and diuretics
140
CLL
| SLL
>60yrs, asymptomatic
cracked chromatin smudge cells, small monomorphic lymphocyte
hypergammaglobulinemia (autoimmu hemolytic anemia)
SLL same as CLL except CLL has increase peripheral blood lymphmocytosis and BM involvement
141
Richter's syndrome
transformation from Cll and Hairy cell leukemia to diffuse large B-cell lymphoma
142
Hairy cell leukemia
adults
B-cell tumor, cell hace filamentous hair like projection
TRAP
treat: cladribine
143
AML
```
65yrs common in chemo
increase myeloblasts: large nuclei, n/c=1, cytoplasmic granules: auer, granule stain with Ab against MPO(myeloperoxidase)
t(15,17)--> M3 AML
respond to all-trans retinoc acid, vit A
M3 commonly present with DIC
```
144
Auer Rod
reddish appearing rod like
peroxidase + cytoplasmic inclusions in granulocytes and myeloblasts
commonly seen in M3 APL
release of Auer Rod--> DIC
145
CML
30-60yrs
myeloid stem cell proliferation increase meut, metamyelocytes, baso
t(9,22), bcr-abl, very low Alka phosph
may accelerate to AML or AMM
responds to imatinib(inhibitor, bcr-abl tyrosine kinase)
146
Langerhans cell histiocytosis
proliferative disorder of DC, functionally immature that cannot stimulate T cell vi APC
Birbeck granules, tennis racket
S-100(neural crest) and CD1a
lytic bone lesion and skin rash
147
JAK2
involved in hematopioetic growth factor signaling
| mutation are implicated in myeloproliferative disorder other than CML
148
JaK2
| itching after hot shower
polycythemia vera
149
decrease plasma volume
| polycythemia
relative poly
150
increase RBC mass
decrease O2 saturation>hypoxia
polycythemia
appropriate absolute
| lung disease, congenital heart disease, high altitude
151
increase RBC mass
polycythe
increase only in RBC
inappropriate absolute
152
increase plasma volume
increase RbC mass
increase in all 3 lines
polycythemia vera
V617 mutation
replace valine with phe more sensitve to GH
proliferate without EPO
