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Year 5 Pathology > Hematology > Flashcards

Hematology Flashcards

1
Q

Factors in extrinsic pathway

A

TF, VII

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2
Q

Factors in intrinsic pathway

A

VIII, IX, XI, XII

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3
Q

Sxs of platelet disorders

A

Bleeding from skin, mucous membranes, petechiae, small superficial ecchymoses.
Immediate bleeding after sx

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4
Q

Sxs coagulation disorders

A

Bleeding into soft tissues, joints, and muscles. Large deep ecchymoses. Delayed bleeding after sx.

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5
Q

Platelet disorders resulting from increased consumption

A

Immune: ITP, drugs

Non-immune: TTp-HUS, DIC

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6
Q

Platelet disorders resulting from decreased platelet fn

A

Inherited: gp Ib mutation; gpIIb/IIIa mutation; storage pool disease
Acquired: Aspirin, uremia

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7
Q

Type I VWD

A

AD. Partial VWF deficiency

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8
Q

Type II VWD

A

AD. Loss multimers-altered quality

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9
Q

Type III VWD

A

AR. Complete deficiency VWF

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10
Q

Presentation VWD

A

Decreased platelet adhesion–>presents as platelet problem.

Increased bleeding time, normal PT, aPTT

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11
Q

Tx-VWD

A

Desmopressin, VIII, VWF concentrates

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12
Q

Clotting derangements in Vit K defic

A

Increased PT and aPTT

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13
Q

Clotting derangements in liver disease

A

Increased PT, aPTT

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14
Q

Protein C or S deficiency

A

AD. Early thrombosis (<40 years)

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15
Q

Prothrombin mutation

A

Increases transcription, therefore more likely to clot

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16
Q

Factor V Lieden

A

Cannot be degraded by protein C

-> Thrombophilia

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17
Q

Causes of acquired thrombophilia

A

Immobility/stasis, tissue trauma (sx), malig, anti-phospholipid syndrome, high estrogen states

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18
Q

Classic signs anemia

A

Sxs hypoxia

Conjunctival pallor, koilonychia, glossitis, angular stomatits, post-cricoid webs, high flow murmur

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19
Q

Causes of cellular bone marrow infiltration

A

Myeloproliferation, myelofibrosis, malignant spread

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20
Q

Causes of hypocellular bone marrow infiltration

A

Idiopathic, cytotoxic drugs +radiation, infxns, AI

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21
Q

Elliptocytosis

A

AD. Mild. No tx

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22
Q

ALL presentation

A

Bone marrow failure, LAD, HSM, testicular infiltration

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23
Q

AML presentation

A

Bone marrow failure, bleeding tendency, GUM/skin/CNS infxns

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24
Q

CLL

A 
Mostly B cell
Elderly
Indolent course with good prognosis
Increased lymphocytes
Smudge cells
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25
Polycythemia rubra vera-presentation and defect
Elderly Non-specific sxs: tired, depressed, vertigo, visual disturbances, HTN, angina, intermittent claudication JAK2 mutation
26
Polycythemia rubra vera-tx
Venesection, hydroxyurea, radioactive 32P
27
Essential thrombocythemia
``` Benign hx Decreased quality platelets so actually bleed Hypercellular marrow JAK2 mutation Tx w/ hydroxyurea ```
28
MGUS
Elderly | Paraprotein present
29
AIP
Psychosis, seizures, abdo pain, polyneuropathy, port wine urine. AD. Precipitated by stress. Tx-analgesia, CHO
30
Bacterial infxn transfusion rxn - Sxs - Tx
Sxs: Sudden onset fever, v unwell. Tx: Stop transfusion, broad spectrum abx
31
Viral infxns possible from blood transfusions
Hep B/C, HIV, CMV, HTLV
32
Graft v host disease-sxs
Skin rashes, organ failure, marrow failure, diarrhea
33
Acanthocytosis
Abetalipoproteinemia, liver disease, hyposplenism
34
basophilic stippling
``` Lead poisoning Megaloblastic anemia Myelodysplasia Liver disease Hemoglobinopathy ```
35
Burr cells (echinocytes)
Uremia, GI bleeidng, stomach carcinoma
36
Heinz bodies
G6PD def, chronic liver disease
37
Leukoerythroblastic anemia
Marrow infiltration, i.e. myelofibrosis, malignancy
38
Pelger Huet cells
Hyposegmented neutrophils
39
Polychromasia
Sign of reticulocytes: premature release from BM
40
Right shift
Megaloblastic anemia, uremia, liver disease
41
Rouleaux formation
Chronic inflamm, paraproteinemia, MYELOMA
42
Schistocytes
MIHA e.g. DIC, HUS, TTP, pre-eclampsia
43
Stomatocytes
"Smiling faces" or "fish mouth" RBCs | Hereditary stomatocytosis, high alcohol intake, liver disease
44
Target cells
``` "HALT" HbC disease Asplenia Liver disease Thalassemia ```
45
Hb components
Heme + globin
46
Heme components
Fe + protoporphyrin
47
Microcytosis
MCV
48
Normocytic anemia
``` MCV 80-100 ACUTE blood loss--first stage IDA Bone marrow failure Renal failure Hypothyroidism Hemolysis Pregnancy F ```
49
Macrocytic anemia
``` MCV>100 Alcohol excess Liver disease B12 defic, folate defic Hypothyroidism (can also be normocytic) ```
50
Anisocytosis
Blood cells of varying size
51
Plummer Vinson syndrome
Anemia Dysphagia (esoph webs) Beefy red tongue
52
Common causes IDA
``` Menorrhagia Peptic ulcers/gastritis Polyps/CRC Meckel's Hookworm infestation Dietary deficiency Malabsorption ```
53
ACD
Fe sequestered by hepcidin in macrophage Increased ferritin, decreased TIBC Decreased Fe, decreased % saturation Causes: - Chronic infection (TB, osteomyelitis) - Vasculitis - RA - Malignancy
54
Sideroblastic anemia
Iron loading causing hemosiderosis Same lab findings as hemochromatosis Causes: Pb poisoning, B6 deficiency (ALAS cofactor), ALAS defect, myeloprolif or myelodysplastic syndromes
55
In which two causes of anemia is Fe low?
IDA, ACD
56
TIBC and ferritin relationship
Oppose each other, i.e. if TIBC high then ferritin low
57
Ferritin
Only low in IDA | Acute phase protein; increases with inflamm e.g. infection, malignancy
58
Megaloblastic blood film
Hypersegmented (>5) neutrophils, leukopenia, macrocytosis, anemia, thrombocytopenia
59
B12 deficiency
Takes years to develop (large liver stores) Diet: Strict vegans, boozers Malabsorption: pernicious anemia, gastrectomy, probs with termina ileum (Crohns, bacterial overgrowth, tropical sprue, diphyllobothrium latum) Pancreatic insufficiency
60
Lab findings ih hemolytic anemias
Increased: unconj bili, urobilinogen, LDH, reticulocytosis/RDW Intravascular types have decreased haptoglobin
61
RBC membrane defect disorders resulting in hemolysis
Hereditary spherocytosis | Hereditary elliptocytosis
62
RBC enzyme defect conditions resulting in hemolytic anemias
G6PD def | PK def
63
Non-immune causes hemolytic anemias
Mechanical heart valves PNH, MAHA Infections (malaria) Drugs
64
Hereditary spherocytosis
``` AD Spectrin, ankyrin, band 3 defect Osmotic fragility test Parvo B19 susceptibility Pigmented gallstones Splenomegaly Tx: splenectomy. Howell-Jolly bodies post-splenectomy ```
65
Hereditary elliptocytosis
AD | Spectrin or band 4.1 mutations
66
G6PD deficiency
X linked African, Mediterranean, Middle Eastern No G6PD=no NADPH (HMP shunt)=reduced glutathione=oxidative injury by H2O2=intravasc hemolysis Oxidants (drugs-malaria drugs, sulfonamides, aspirin; fava beans; stress) cause rapid anemia and jaundice Bite cells and Heinz bodies Enzyme assay 2-3 months after attack Intarvasc hemolysis: dark urine
67
Pyruvate kinase deficiency
AR | Severe neonatal jaundice, splenomegaly, hemolytic anemia
68
Sickle cell
AR Glu ->valine position 6 of beta chain Trait usu asymptomatic (except kidneys) Manifests around 6mo (HbF as newborn) Decreased O2 tension causes polymerization and sickling Get vaso-occlusion and infarction: dactylitis, stroke, acute chest syndrome, renal papillary necrosis Tx: analgesia, hydration, SHiN vaccines, hydroxyurea
69
Beta thal minor
Asymptomatic carrier, mild anemia
70
Beta thal intermedia
Moderate anemia, splenomegaly, bony deformity, gallstones
71
Beta thal major
Severe anemia, FTT, HSM (extramedullary hematopoiesis), bony deformity. May have heart failure
72
2 alpha gene deletion
Alpha thal trait | Asymptomatic, mild anemia
73
3 alpha gene deletion
HbH disease | Moderate anemia, splenomegaly
74
4 alpha gene deletion
Hb Barts | Incompatible with life
75
Warm Agglutinin Autoimmune Hemolytic Anemia
IgG Coombs positive Cause: mainly idiopathic, may be lymphoma, CLL, SLE, methydopa Tx: steroids, splenectomy, immunosuppression
76
Cold Agglutinin-Autoimmune Hemolytic Anemia
IgM Positive Coombs test Oft has Raynauds Causes: primarily idiopathic. may be lymphoma, infections (EBV, mycoplasma pneumoniae) Tx: treat underlying condition, avoid cold, chlorambucil
77
Paroxysmal cold hemoglobinuria
Hb-uria after viral infection (e.g. measles) | Donath-Landsteiner abs attach to RBCs in cold; complement-mediated hemolysis on rewarming
78
Paroxysmal Nocturnal Hemoglobinuria
Acquired loss GPI on RBC GPI anchors DAF which protects against complement in blood. If you lose GPI, you lose DAF, therefore complement-mediated lysis Dx: Hamm's test or immunophenotype showing altered GPI
79
Where in gut is iron absorbed?
Duodenum
80
Transferrin
Transports iron in blood.
81
Ferritin
Binds iron in storage | Note that Fe on its own causes ROS damage via Fenton reaction
82
TIBC
Measure of transferrin molecules in blood
83
% saturation
Percentage of transferrin molecules that are bound by Fe (normal is 33%)
84
Most common type of anemia in hospitalized patients
ACD
85
Hepcidin
Sequesters Fe in storage sites by: 1. Limiting transfer of Fe from macros to erythroid precursors 2. Suppressing EPO
86
lab findings in ACD
Increased ferritin, decreased TIBC Decreased serum Fe and % saturation Increased FEP
87
Lab findings in IDA
Decreased ferritin, increased TIBC Decreased serum iron, decreased % saturation Increased FEP
88
HbF
a2g2
89
HbA
a2b2
90
HbA2
a2delta2
91
Causes macrocytic anemia without megaloblastic change
Booze Liver disease Drugs (e.g. 5-FU)
92
Where is folate absorbed?
Jejunum
93
Difference in labs between folate and B12 deficiency
Folate: normal methylmalonic acid B12: increased methylmalonic acid Both have high homocysteine (increases risk thrombosis)
94
Corrected retic count
(Retic count falsely elevated in anemia, as normal RBCs gone so retics are overall higher percentage) Retic count * Hct/45
95
Extravascular hemolysis
Destruction by reticuloendothelial system (macros of spleen, liver, LNs) Findings: Anemia, splenomegaly, unconj jaundice, risk bili gallstones Marrow hyperplasia if corrected retic count >3%
96
Intravascular hemolysis
Hb-emia Hb-uria Hemosiderinuria (renal tubular cells pick up Hb and break down to Fe, accumulates and shed with tubular cells) Decreased haptoglobin
97
Microangiopathic hemolytic anemia (MIHA)
Intravasc hemolysis from VASCULAR cause...RBCs destroyed as pass through circulation. Caused by microthrombi (TTP-HUS, HIC, HELLP), prosthetic heart valves, aortic stenosis Schistocytes
98
Fever patterns: - Falciparum - Vivax - Ovale - malariae
Falcip: every day Vivax, ovale: tertian (every other day) Malariae: quartan (every third day)
99
Aplastic anemia
Damage to hematopoietic stem cells (CD34+) cauing pancytopenia with low retic count Causes: drugs/chemicals, viral infections, AI damage Biopsy: empty, fatty marrow
100
Primary hemostasis
Formation of platelet plug
101
Secondary hemostasis
Stabilization platelet plug | Coag cascade mediated
102
Mucosal bleeding
Epistaxis (most comm), hemoptysis, GI bleeding, hematuria, menorrhagia Symptom of platelet abnormality
103
Petechiae vs purpura vs ecchymoses
Petechia: 1-2mm Purpura: >3mm Ecchymoses: >1cm NB petechiae are a sign of thrombocytopenia, not usu seen with qualitative platelet defects (just quantitative)
104
ITP
AI production IgG against platelet antigens, e.g. GpIIb/IIIa MCC thrombocytopenia in adults Ab-bound platelets consumed by splenic macros causing thrombocytopenia Acute: post viral infection or immunization. Kids. Self-limiting Chronic: primary or secondary. Women of child bearing age
105
TTP-HUS
TTP: ADAMTS13 deficiency HUS: E. coli O157:H7 verotoxin destroys endothelial cells resulting in platelet microthrombi ``` "FATRN" Fever Anemia (MIHA) Thrombocytopenia Renal failure Neuro sxs ```
106
Bernard-Soulier
GpIb deficiency--impaired platelet adhesion | Mild thrombocytopenia with enlarged platelets
107
Glanzmann thrombasthenia
GpIIb/IIIa deficiency--impaired platelet aggregation
108
Uremia's effect on platelets
Disrupts adhesion and aggregation of platelets
109
Clinical difference between hemophilia A and B
There is none, you numpty
110
Vitamin K deficiency--causes
Newborn (lack GI colonization) Long term antibiotic therapy Malabsorption of fat-soluble vitamins
111
Heparin-Induced Thrombocytopenia
Platelet destruction. | Fragments destroyed platelets may activate others, causing thrombosis
112
DIC
Widespread microthrombi result in ischemia and infarction Consumption factors and platelets result in bleeding, esp from IV sites and mucosal surfaces ``` Causes: Obstetric complications--tissue thromboplastin in amniotic fluid activates coag Sepsis, esp E. coli and N men Adenocarcinoma (mucin activates coag) APML Rattlesnake bites ```
113
Lab findings in DIC
Decreased: platelets, fibrinogen, D-dimer Increased: PT, PTT
114
tPA
Converts plasminogen to plasmin
115
Plasmin
Cleaves fibrin and serum fibrinogen, destroys coag factors, blocks platelet activation
116
How do you distinguish thrombosis from postmortem clot?
Lines of Zahn Attachment to vessel wall (Both these found in thrombosis)
117
Mechanisms by which intact endothelium prevents thrombosis
Block exposure subendothelial collagen and underlying tissue factor Produce PGI2 and NO (vasodilation and inhibition platelet agg) Secrete heparin-like molecules Secrete tPA Secrete thrombomodulin
118
Causes endothelial cell damage :(
Atherosclerosis Vasculitis High levels homocysteine
119
Cystathionine beta synthase deficiency
Results in high levels homocysteine and homocystinuria. | Findings: vessel thrombosis, mental retardation, lens dislocation, long slender fingers
120
Protein C or S deficiency
AD | Increased risk warfarin skin necrosis
121
Most common cause inherited hypercoagulable state
Factor V Lieden
122
Prothrombin mutation
Point mutation in thrombin that causes increased gene expresion, therefore more thrombi (pro-thrombin...)
123
ATIII deficiency
Decreases protective effect of heparin-like molecules produced by endothelium
124
Why does estrogen (e.g. OCP) cause increased thrombosis risk?
Increased production coag factors
125
Types of emboli
Thrombo-embolism (MCC) Fat embolism Gas embolism Amniotic fluid embolism
126
Fat embolus
BONE FRACTURES, esp long bones, and soft tissue trauma | Dyspnea and petechiae on skin over chest
127
Gas embolus
Decompression sickness--rapid ascent while diving Nitrogen gas precipitates out during rapid ascent Joint and muscle pains (the bends) and resp sxs (the chokes) Chronic form=Caisson disease. Multifocal ischemic necrosis of bone May also occur during laparoscopic surgery
128
Amniotic fluid embolism
Amniotic fluid enters maternal circulation during labor/delivery SOB, neuro sxs, DIC Squamous cell and keratin debris from fetal skin found in thrombus Extremely high mortality: only 10% survive
129
Pulmonary embolism
Most often clinically silent due to dual blood supply of lung Usually small and self-resolves 10% cause infarction: SOB, hemoptysis, pleuritic CP, pleural effusion Sudden death if saddle embolus Pulm HTN if chronic emboli that are reorganized over time
130
Systemic embolism
Usu due to thromboembolus | Most commonly arise in L heart
131
factor II
Aka thrombin
132
JAK 2
Non-receptor tyrosine kinase ``` Mutations found in: PV ET Myelofibrosis Other Myeloproliferative disorders ```
133
Acquired causes sideroblastic anemia
Alcoholism, Pb poisoning, B6 deficiency
134
Disorders with increased MCHC
Hereditary spherocytosis | AI anemia
135
Metabisulfite test
Detects both sickle cell and sickle cell trait, i.e. any presence of HbS
136
HbC disse
LYSINE instead glutamic acid HbC crystals
137
PNH-cause
ACQUIRED deficiency GPI
138
GPI
Anchors DAF and MIRL which degrade complement getting too close to RBCs
139
Test for PNH
Look for presence CD55 (aka DAF) on cells. If no CD55, then can assume no GPI
140
MCC death in PNH
Thrombosis, e.g. portal vein | 10% go on to develop AML
141
HIT
Heparin destroys platelets. Some of destroyed platelets go on to activate other platelets causing THROMBOSIS Do not use warfarin--increased incidence skin necrosis
142
Secondary causes DIC
Obstetric complications (amniotic fluid activates tissue thromboplastin), sepsis, adenocarcinoma, APML (Auer rods), rattlesnake bite
143
GpIb-function
Binds vWF
144
GpIIb/IIIa
Platelet AGGREGATION. Uses fibrinogen as linker
145
Petechiae are a sign of what problem?
QUANTITATIVE platelet problem, i. thrombocytopenia. Not seen in qualitative disorders
146
ITP
AI: IgG against platelet antigens (eg. GpIIb/IIIa). Most common cause thrombocytopenia in kids and adults Ab-bound platelets consumed by splenic macrophages Spleen also one making antibody Acute-kids. Usu post-viral infxn or immunization Chronic-adult women (may be secondary to SLE) Tx: steroids (esp if acute), IVIG, splenectomy
147
MIHA
Pathological formation microthrombi in small vessels, which shears RBCs. Use up platelets making microthrombi TTP-HUS is a major cause
148
TTP-mechanism
Deficiency ADAMTS13--can't degrade vWF multimers. Pile up vWF multimers causes abn platelet adhesion thus microthrombi
149
HUS-mechanism
E. coli O157:H7 vertotoxin damages endotherlial cells causing platelet microthrombi. Also decreases ADAMTS13 Kids + undercooked beef -->dysentery
150
Signs and sxs TTP-HUS
``` Fever MIHA Thrombocytopenia Renal insufficiency (esp HUS) CNS abnormalities (esp TTP) ``` Increased bleeding time, normal PT/PTTtX-ttp-hus
151
Tx-TTP-HUS
Plasmapheresis, 'roids
152
Bernard-Soulier
Genetic gpIb deficiency Impaired platelet adhesion Mild thrombocytopenia with enlarged platelets ("Big Suckers")
153
Glanzmann's thrombasthenia
Deficienct gpIIb/IIIa | Impaired platelet aggregation
154
Indications for transfusion
Acute blood loss >30% blood volume | Peri-op/critical care, when Hb
155
Intra operative cell salvage
Collect blood lost during surgery and reinfuse into pt. | NB-washed/filtered so lose coagulation factors and platelets
156
Type of blood used in transfusions for highly immunosuppressed individuals
Irradiated
157
Reaction when use the wrong blood in transfusion
Signs/sxs Intravascular hemolysis | Restless, chest/loin pain, fever, vomiting, flushing, collapse, later--hemoglobinuria
158
Febrile non-hemolytic transfusion reaction
Increase in temp by 1 degree C, chills, rigors Common before blood leukodepleted, now rare Stop/slow transfusion, give paracetamol. Can re-start
159
Allergic transfusion reaction
Usually with plasma Common Mild urticarial or itchy rash +/- wheeze Tx with antihistamines
160
Delayed hemolytic transfusion reaction
1-3% pts who have received transfusion Develop Sb to one of foreign RBC antigens Complement-mediated phagocytosis Extravascular hemolysis
161
Anaphylactic reaction to blood transfusion
Hypotensive shock, very breathless, wheeze, laryngeal and facial edema E.g. Donor eaten peanuts (antigen) and pt has IgE Ab to this antigen already
162
TACO
Transfusion Associated Circulatory Overload Within 6 hrs Acute resp distress, tachycardia, increased BP, acute or worsening pulm edema, positive fluid balance
163
TRALI
Transfusion Related Acute Lung INjury Dry cough, dyspnea, fever, not due to fluid overload or other causes Donor anti-leukocytic Abs interact with pt's leukocyte antigens causing aggregation. These aggregations get stuck in pulm caps. Neutrophils in aggregates release proteolytic enzymes that cause lung damage Can prevent by giving virally-activated plasma, and plasma from men
164
Cabot rings
Looped structures in RBCs. Found in megaloblastic anemia
165
Pappenheimer bodies
Iron granules. Foud in sideroblastic anemia, Pb poisoning, hemolytic anemias
166
Prothrombin 20210A mutation
Substitution G to A at position 20210 of prothrombin gene. Increases transcription thus more clotting after vessel damage 5% Caucasian population
167
Post-transfusion purpura
5-9 days after transfusion Severe thrombocytopenia with bleeding Tx with IVIG
168
Pencil bodies
Type of elliptocyte | Seen in IDA, thal, PK deficiency
169
Cooley's anemia
Old name for beta thal major
170
Cabot rings
Looped structures in RBCs | Caused by megaloblastic anemia
171
Pappenheimer bodies
Iron granules. Pb poisoning, sideroblastic anemia, hemolytic anemia
172
Buerger's disease
Aka thromboangitis obliterans Strong relation to smoking Recurrent arterial and venous thromboses Corkscrew arteries in upper and lower limbs
173
Protein S
Degrades Factors Va, VIIIa
174
Antithrombin
Inhibits thrombin, factor Xa
175
C-myc
Burkitts
176
Lymphoma with centrocytes, centroblasts
Follicular
177
Bcl-2 associated lymphoma
Follicular
178
Donath-Landsteiner Abs
Paroxysmal cold Hb-uria
179
Non-megaloblastic causes of macrocytic anemia
``` "RALPH" Reticulocytosis Alcohol Liver disease Pregnancy? Hypothyroidism ```
180
Hemochromatosis-sxs
``` Hepatomegaly/deranged LFTs Arthralgia and chondrocalcinosis Diabetes DCM Hypogonadism/impotence Adrenal insufficiency Skin color changes (slate grey or bronze) ```
181
MM-lab findings
Normal ALP Increased ESP Increased Ca
182
MGUS
183
Waldenstroms
High IgM | Hyperviscosity and vascular complications
184
Combined polycythemia
Aka "smoker's polycythemia" Combination decreased plasma volume and decreased O2 (CO displaces) causing an increase in EPO Be very suspicious if smoker + COPD picture
185
Secondary polycythemia
Increase in EPO or testosterone (anabolic roids) | Ectopic EPO from HCC, RCC, hemangioblastoma, pheo, uterine myomata
186
Primary polycythemia
LOW EPO | E.g. polycythemia vera
187
Non-classical subtype of Hodgkins
Nodular lymphocytic
188
Ann Arbor Staging for Hodgkins
I: Single LN region II: 2+ LN on SAME SIDE of diaphragm III: LN on both sides diaphragm IV: extranodal spread Add "a" if no B sxs, add "B" if there are B sxs
189
B sxs
Significant unexplained fever Night sweats Weight loss >10% in preceding 6/12
190
Imantinib
Tyrosine kinase inhibitor. | Used in CML
191
Absence LONG arm chromo 5
Subtype of myelodysplastic syndrome
192
Absence SHORT arm chromo 5
Cri-du-chat | They will try to trick you with long v short arm

Year 5 Pathology (6 decks)

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