Heme Disease Flashcards
1
Q
Leukopenia: Signs/Dx Criteria AND Pathophysiology
A
WBC < 3,000
2
Q
Leukopenia: Type
A
Leukocyte Disorders
3
Q
Neutropenia: Signs/Dx Criteria AND Pathophysiology
A
ANC < 1,500
4
Q
Neutropenia: Type
A
Leukocyte Disorders
5
Q
Neutropenia: Etiologies
A
Infection, medications, blood cancers, autoimmune, inherited, ethnic/benign
6
Q
Neutropenia: Treatment
A
Filgrastim
7
Q
Lymphopenia: Signs/Dx Criteria AND Pathophysiology
A
Low lymphocytes
8
Q
Lymphopenia: Type
A
Leukocyte Disorders
9
Q
Lymphopenia: Etiologies
A
HIV, EBV, CMV, steroids, immunosuppressants, chemo, Hodgkin’s
10
Q
Neutrophilia: Signs/Dx Criteria AND Pathophysiology
A
High neutrophils
11
Q
Neutrophilia: Type
A
Leukocyte Disorders
12
Q
Neutrophilia: Etiologies
A
Infection, steroids, autoimmune, leukemoid reaction, blood cancers
13
Q
Eosinophilia: Signs/Dx Criteria AND Pathophysiology
A
High eosinophils
14
Q
Eosinophilia: Type
A
Leukocyte Disorders
15
Q
Eosinophilia: Etiologies
A
“NAACP” - Neoplasia, Allergy, Addison’s Dz, Collagen Vascular Dz, Parasites
16
Q
Pelger-Huët Abnormality: Epi
A
AD
17
Q
Pelger-Huët Abnormality: Signs/Dx Criteria
A
Hyposegmented PMNs
18
Q
Pelger-Huët Abnormality: Type
A
Leukocyte Disorders
19
Q
Pelger-Huët Abnormality: Pathophysiology
A
Lamin B receptor (LBR) gene defect
20
Q
May-Hegglin Anomaly: Epi
A
AD
21
Q
May-Hegglin Anomaly: Signs/Dx Criteria
A
Macrothrombocytopenia
22
Q
May-Hegglin Anomaly: Type
A
Leukocyte Disorders AND Bleeding Disorders
23
Q
May-Hegglin Anomaly: Pathophysiology
A
Non-muscle myosin heavy chain IIA (MYH9) gene defect, Myosin mutation
24
Q
May-Hegglin Anomaly: Etiologies
A
Rare inherited
25
May-Hegglin Anomaly: Treatment
???
26
Chediak-Higashi Syndrome: Epi
AR
27
Chediak-Higashi Syndrome: Signs/Dx Criteria
WBC dysfunction
28
Chediak-Higashi Syndrome: Type
Leukocyte Disorders
29
Chediak-Higashi Syndrome: Pathophysiology AND Etiologies AND Treatment
Impaired fusion of phagosome and lysosome
30
Chronic Granulomatous Disease: Epi
XLR/AR
31
Chronic Granulomatous Disease: Signs/Dx Criteria
Granulomas, ↑macrophages
32
Chronic Granulomatous Disease: Type
Leukocyte Disorders
33
Chronic Granulomatous Disease: Pathophysiology AND Etiologies AND Treatment
NADPH oxidase defect → inadequate phagolysosome activity
34
Bruton's Agammaglobulinemia: Epi
XLR
35
Bruton's Agammaglobulinemia: Signs/Dx Criteria
Complete lack of antibodies
36
Bruton's Agammaglobulinemia: Type
Leukocyte Disorders
37
Bruton's Agammaglobulinemia: Pathophysiology AND Etiologies AND Treatment
Bruton's tyrosine kinase (Btk) gene defect → defective B cell development
38
Severe Combined Immunodeficiencies: Epi
XLR/AR
39
Severe Combined Immunodeficiencies: Signs/Dx Criteria
↓B and T cells, ↑↑urine deoxyadenosine concentration
40
Severe Combined Immunodeficiencies: Type
Leukocyte Disorders
41
Severe Combined Immunodeficiencies: Pathophysiology AND Etiologies AND Treatment
↓Adenosine deaminase → toxic amounts of dATP, inhibition of nucleotide synthesis
42
Megaloblastic Anemia: Symptoms
Neurological symptoms (B12 only), achlorhydria if pernicious
43
Megaloblastic Anemia: Signs/Dx Criteria
Hypersegmented neutrophils, oval macrocytes, megaloblasts on bone marrow smear, ↑LDH, pancytopenia, ↑homocysteine
44
Megaloblastic Anemia: Type
Iron Metabolism Disorders
45
Megaloblastic Anemia: Pathophysiology
DNA replication leads to marrow failure
46
Megaloblastic Anemia: Etiologies
B12 (cobalamin)/folic acid deficiency, or pernicious (autoimmune vs. parietal cells)
47
Megaloblastic Anemia: Treatment
B12 + folate replacement therapy, avoid transfusions unless hemodynamic compromise or angina
48
Iron Deficiency Anemia: Epi
Pregnant women
49
Iron Deficiency Anemia: Symptoms
Fatigue
50
Iron Deficiency Anemia: Signs/Dx Criteria
Pica, atrophic stomatitis, esophageal web, koilonychias, lack of dark iron on marrow stain, hypochromic
51
Iron Deficiency Anemia: Type
Iron Metabolism Disorders
52
Iron Deficiency Anemia: Pathophysiology
Problem in DMT-1 or HFE
53
Iron Deficiency Anemia: Etiologies
Malnutrition or genetic
54
Iron Deficiency Anemia: Treatment
Iron replacement therapy (ferrous sulfate +/- vit C, parenteral)
55
Anemia of Chronic Disease: Signs/Dx Criteria
Mild, non-progressive anemia, normochromic + normocytic RBCs,
56
Anemia of Chronic Disease: Type
Iron Metabolism Disorders
57
Anemia of Chronic Disease: Pathophysiology
↑hepcidin w/ inflammation, sequesters ferroportin, leading to ↓iron from macs, ↓GI iron absorption
58
Anemia of Chronic Disease: Etiologies
CVD, IBD, chronic infxn, malignancy, renal failure, thyroid dz, familial Mediterranean fever
59
Anemia of Chronic Disease: Treatment
Treat underlying cause
60
Hemochromatosis: Epi
N. Europeans
61
Hemochromatosis: Signs/Dx Criteria
Darkened skin + endocrinopathies ("bronzing diabetes"), arrhythmias, RCM, cirrhosis, liver cancer, arthritis, testicular atrophy, ↑transferrin
62
Hemochromatosis: Type
Iron Metabolism Disorders
63
Hemochromatosis: Pathophysiology
Too much iron absorption (DMT-1) or too little iron excretion (HFE)
64
Hemochromatosis: Etiologies
Genetic DMT-1/HFE defect (autosomal dominant) or excess transfusions
65
Hemochromatosis: Treatment
Phlebotomy, iron chelation, liver transplant, gene therapy?
66
Hereditary Spherocytosis: Epi
10-15 yo, 1:2K-5K in Europe
67
Hereditary Spherocytosis: Symptoms
Splenomegaly, malleolar ulcers, cholelithiasis
68
Hereditary Spherocytosis: Signs/Dx Criteria
RBCs smaller, no central pallor; chronic anemia, ↑osmotic fragility, direct Coombs(-), ↑bilirubin
69
Hereditary Spherocytosis: Type
Congenital Hemolytic Anemias
70
Hereditary Spherocytosis: Pathophysiology
Mutation in ankyrin/spectrin = distorted shape
71
Hereditary Spherocytosis: Etiologies
AD congenital mutation in proteins associated with spectrin → release of lipid membrane vesicles
72
Hereditary Spherocytosis: Treatment
Folate, transfusions, splenectomy (curative)
73
Hereditary Elliptocytosis: Symptoms
Splenomegaly, malleolar ulcers, cholelithiasis
74
Hereditary Elliptocytosis: Signs/Dx Criteria
Elliptical RBCs +/- no central pallor
75
Hereditary Elliptocytosis: Type
Congenital Hemolytic Anemias
76
Hereditary Elliptocytosis: Pathophysiology
Mutation in ankyrin/spectrin = distorted shape
77
Hereditary Elliptocytosis: Etiologies
AD congenital mutation in proteins associated with spectrin → release of lipid membrane vesicles
78
Hereditary Elliptocytosis: Treatment
Folate, transfusions, splenectomy (curative)
79
G6PD Deficiency: Epi
(A-): Africa, (B-): Medit.
80
G6PD Deficiency: Symptoms
Worsening with infection, drugs
81
G6PD Deficiency: Signs/Dx Criteria
Heinz bodies on supravital stain of smear, extravascular anemia, direct Coombs(-), hemolysate testing (unreliable if right after hemolyic episodes from ↑retic), PCR
82
G6PD Deficiency: Type
Congenital Hemolytic Anemias
83
G6PD Deficiency: Pathophysiology
Deficiency of G6PD or others in HMP shunt pathway → ↓GSH, RBC lysis from ↑oxidative damage
84
G6PD Deficiency: Etiologies
XLR congenital mutation (Gd gene on Xq28), worsened by infxn, drugs
85
G6PD Deficiency: Treatment
Supportive, avoid oxidative stresses (infection, drugs)
86
PK Deficiency: Type
Congenital Hemolytic Anemias
87
PK Deficiency: Pathophysiology AND Etiologies
EB pathway abnormality
88
HbE: Type
Congenital Hemolytic Anemias
89
HbE: Pathophysiology AND Etiologies
↓Beta chain production
90
Hb Lepore: Type
Congenital Hemolytic Anemias
91
Hb Lepore: Pathophysiology AND Etiologies
Beta-delta chain fusion
92
Hb Koln: Type
Congenital Hemolytic Anemias
93
Hb Koln: Pathophysiology AND Etiologies
↓Hemoglobin stability
94
Paroxysmal Nocturnal Hemoglobinuria (PNH): Epi
M=F, 20-50 yo
95
Paroxysmal Nocturnal Hemoglobinuria (PNH): Symptoms AND Signs/Dx Criteria
↑clotting, intermittent dark urine, renal disease, direct Coombs(-), intravascular hemolysis
96
Paroxysmal Nocturnal Hemoglobinuria (PNH): Type
Acquired Hemolytic Anemias
97
Paroxysmal Nocturnal Hemoglobinuria (PNH): Pathophysiology
Can't stop complement cascade
98
Paroxysmal Nocturnal Hemoglobinuria (PNH): Etiologies
↓CD55/59 from PIGA mutation
99
Paroxysmal Nocturnal Hemoglobinuria (PNH): Treatment
Iron, folate, transfusions, eculizumab, anti-coag
100
Mechanical Hemolytic Anemia: Signs/Dx Criteria
Schistocytes
101
Mechanical Hemolytic Anemia: Type
Acquired Hemolytic Anemias
102
Mechanical Hemolytic Anemia: Pathophysiology AND Etiologies
Heart valve, TTP, or DIC physically damages RBCs
103
Infection/Venom-related: Type
Acquired Hemolytic Anemias
104
Infection/Venom-related: Pathophysiology AND Etiologies
Infection/venom damages blood cells
105
Spur Cell Anemia: Type
Acquired Hemolytic Anemias
106
Spur Cell Anemia: Etiologies
Liver disease
107
Hemolytic Transfusion Reactions (HTR): Symptoms AND Signs/Dx Criteria
Anemia
108
Hemolytic Transfusion Reactions (HTR): Type
Acquired Hemolytic Anemias
109
Hemolytic Transfusion Reactions (HTR): Pathophysiology AND Etiologies
Donor RBCs destroyed by pt antibodies
110
Hemolytic Disease of Newborn (HDN): Symptoms AND Signs/Dx Criteria
Anemia with eryhtroblastosis, direct Coombs(+)(IgG), fetal heart failure/hydrops fetalis, post-delivery jaundice, kernicterus
111
Hemolytic Disease of Newborn (HDN): Type
Acquired Hemolytic Anemias
112
Hemolytic Disease of Newborn (HDN): Pathophysiology
Mom has IgG to baby's RBCs (ABO mild, Rh severe)
113
Hemolytic Disease of Newborn (HDN): Etiologies
In Rh, affects 2nd/3rd baby (since 1st baby = first exposure to D-antigen
114
Hemolytic Disease of Newborn (HDN): Treatment
Anti-Rh Ig prophylaxis, intrauterine transfusion, phototherapy if mild
115
Warm Autoimmune Hemolytic Anemia (WAIHA): Epi
Middle-age women (idiopathic)
116
Warm Autoimmune Hemolytic Anemia (WAIHA): Symptoms AND Signs/Dx Criteria
↑clotting, warm Ab screen, direct Coombs(+) in 90% (IgG ± C3d), microspherocytes, pallor, scleral icterus, splenomegaly > hepatmoegaly, thromboembolism
117
Warm Autoimmune Hemolytic Anemia (WAIHA): Type
Acquired Hemolytic Anemias
118
Warm Autoimmune Hemolytic Anemia (WAIHA): Pathophysiology
Autoimmune IgG (80% of AIHAs)
119
Warm Autoimmune Hemolytic Anemia (WAIHA): Etiologies
55% idiopathic, 45% 2ndary to autoimmune / lymphoproliferative dz
120
Warm Autoimmune Hemolytic Anemia (WAIHA): Treatment
Steroids, splenectomy, anti-coag, rituximab
121
Cold Autoimmune Hemolytic Anemia (CAIHA): Symptoms AND Signs/Dx Criteria
Cold Ab screen(+), direct Coombs(+)(C3d), ↑bilirubin, normocytic normochromic anemia w/ poikilocytosis
122
Cold Autoimmune Hemolytic Anemia (CAIHA): Type
Acquired Hemolytic Anemias
123
Cold Autoimmune Hemolytic Anemia (CAIHA): Pathophysiology
Autoimmune IgM (20% of AIHAs)
124
Cold Autoimmune Hemolytic Anemia (CAIHA): Etiologies
Usually 2ndary to CMV, HIV, EBV, mycoplasma
125
Cold Autoimmune Hemolytic Anemia (CAIHA): Treatment
Plasma exchange, rituximab
126
Paroxysmal Cold Hemoglobinuria (PCH): Signs/Dx Criteria
Donath-Landsteriner antibody
127
Paroxysmal Cold Hemoglobinuria (PCH): Type
Acquired Hemolytic Anemias
128
Paroxysmal Cold Hemoglobinuria (PCH): Pathophysiology
Rare IgG cold-acting antibody
129
Paroxysmal Cold Hemoglobinuria (PCH): Etiologies
Post-varicella/viral in children
130
Drug-Related Hemolytic Anemia: Type
Acquired Hemolytic Anemias
131
Drug-Related Hemolytic Anemia: Pathophysiology AND Etiologies AND Treatment
Mechanisms: Haptenic (IgG to drug binds RBC), drug-protein neoantigen (IgG, IgM, complement), non-specific Ig adhesion (hemolysis rare), induction of autoimmunity (continues even after drug stopped)
132
Alpha Thalassemia: Epi
Africa, Asia
133
Alpha Thalassemia: Symptoms
Anemia, hemolysis
134
Alpha Thalassemia: Signs/Dx Criteria
Microcytic anemia, hemolysis, hydrops fetalis if 0/4 alleles; microcytosis, hypochromia, aniso-/poikilocytosis, +/- target cells, lots of pallor in β
135
Alpha Thalassemia: Type
Hemoglobin Disorders
136
Alpha Thalassemia: Pathophysiology
3/4 alleles nl = silent carrier, 2/4 = trait, 1/4 = major, 0/4 = lethal
137
Alpha Thalassemia: Etiologies
Congenital gene deletion
138
Alpha Thalassemia: Treatment
Transfusions + iron chelators, bone marrow transplant, gene therapy?
139
Beta Thalassemia: Epi
Mediterran.
140
Beta Thalassemia: Symptoms
Anemia, hemolysis
141
Beta Thalassemia: Signs/Dx Criteria
Microcytic anemia, hemolysis, hydrops fetalis if 0/4 alleles; microcytosis, hypochromia, aniso-/poikilocytosis, +/- target cells, lots of pallor in β
142
Beta Thalassemia: Type
Hemoglobin Disorders
143
Beta Thalassemia: Pathophysiology
Heterozygote = β+, homozygote = β0 (major); ↓β, ↑α
144
Beta Thalassemia: Etiologies
Congenital point mutation, ↓expression
145
Beta Thalassemia: Treatment
Transfusions + iron chelators, bone marrow transplant, gene therapy?
146
Sickle Cell Anemia : Symptoms
Bone pain, anemia, priapism
147
Sickle Cell Anemia : Signs/Dx Criteria
Hematuria, hyposthenuria, anemia, painful (occlusive) crises, pulm HTN, stroke, malleolar ulcers, splenic sequestration/infarct, target cells in HbC
148
Sickle Cell Anemia : Type
Hemoglobin Disorders
149
Sickle Cell Anemia : Pathophysiology
Deoxyhemoglobin mutated so it easily polymerizes, shape change, stuck in post-capillary venules
150
Sickle Cell Anemia : Etiologies
Congenital HbS (glu6val) or HbC (glu6lys) mutation
151
Sickle Cell Anemia : Treatment
Hydroxyurea (shifts to HbF producing stem cells) transfusions + iron chelators, bone marrow transplant
152
Hemophilia A: Epi
XLR pattern, women w/ extreme Lyonization
153
Hemophilia A: Symptoms
Bleeding
154
Hemophilia A: Signs/Dx Criteria
Bleeding (macrohemorrhage, cerebral, soft tissue, hematuria, GI, surgical, epistaxis, hemarthroses), easy bruising, ↑PTT
155
Hemophilia A: Type
Bleeding Disorders
156
Hemophilia A: Pathophysiology
Factor 8 deficiency
157
Hemophilia A: Etiologies
XLR inherited
158
Hemophilia A: Treatment
Prophylactic recombinant factor replacement
159
Hemophilia B: Epi
XLR pattern, women w/ extreme Lyonization
160
Hemophilia B: Symptoms
Bleeding
161
Hemophilia B: Signs/Dx Criteria
Bleeding (macrohemorrhage, cerebral, soft tissue, hematuria, GI, surgical, epistaxis, hemarthroses), easy bruising, ↑PTT
162
Hemophilia B: Type
Bleeding Disorders
163
Hemophilia B: Pathophysiology
Factor 9 deficiency
164
Hemophilia B: Etiologies
XLR inherited
165
Hemophilia B: Treatment
Prophylactic recombinant factor replacement
166
Hemophilia C: Epi
XLR pattern, women w/ extreme Lyonization
167
Hemophilia C: Symptoms
Bleeding
168
Hemophilia C: Signs/Dx Criteria
↑PT, Discovered during surgery
169
Hemophilia C: Type
Bleeding Disorders
170
Hemophilia C: Pathophysiology
Factor 11 deficiency
171
Hemophilia C: Etiologies
AR inherited
172
Hemophilia C: Treatment
Fresh frozen plasma
173
Von Willebrand's Disease: Epi
O blood type
174
Von Willebrand's Disease: Symptoms
Bleeding
175
Von Willebrand's Disease: Signs/Dx Criteria
Menorrhagia, easy bruising, dental + surgical bleeding, no hemarthroses, +/- ↑PTT
176
Von Willebrand's Disease: Type
Bleeding Disorders
177
Von Willebrand's Disease: Pathophysiology
vWF deficiency, defect of platelet adhesion, ↓factor 8
178
Von Willebrand's Disease: Etiologies
AD, variable penetrance; most common bleeding disorder
179
Von Willebrand's Disease: Treatment
Aminocaproic acid, desmopressin, humate-P, cryoprecipitate
180
Vitamin K Deficiency: Symptoms
Bleeding
181
Vitamin K Deficiency: Signs/Dx Criteria
↑PT, ↑PTT
182
Vitamin K Deficiency: Type
Bleeding Disorders
183
Vitamin K Deficiency: Pathophysiology
↓production of factor 2, 7, 9, 10, C, S
184
Vitamin K Deficiency: Etiologies
Malnutrition, antibiotics
185
Vitamin K Deficiency: Treatment
Replace vitamin K
186
Immune Thrombocytopenic Purpura: Epi
Young woman
187
Immune Thrombocytopenic Purpura: Signs/Dx Criteria
Very few huge platelets on smear, microhemorrhage, mucous membrane bleeding, epistaxis, non-blanching petechiae, purpura
188
Immune Thrombocytopenic Purpura: Type
Bleeding Disorders
189
Immune Thrombocytopenic Purpura: Pathophysiology
↓platelets due to Ab against them
190
Immune Thrombocytopenic Purpura: Etiologies
Can be idiopathic, secondary to HIV, etc., first rule out other causes
191
Immune Thrombocytopenic Purpura: Treatment
1st line: Steroids, IVIg, IV anti-D. 2nd line: splenectomy, rituximab, thrombopoietin mimics
192
Uremic Platelet Dysfunction: Type
Bleeding Disorders
193
Uremic Platelet Dysfunction: Pathophysiology AND Etiologies
Renal failure causes endo + PLT dysfunction
194
Uremic Platelet Dysfunction: Treatment
Dialysis, desmopressin
195
Poisoned Platelets: Type
Bleeding Disorders
196
Poisoned Platelets: Pathophysiology
Drugs ↓stickiness
197
Poisoned Platelets: Etiologies
ASA, NSAIDs, ADPr inhibitors, alcohol
198
Medication-Induced Thrombocytopenia: Type
Bleeding Disorders
199
Medication-Induced Thrombocytopenia: Pathophysiology
Antibody formation vs. drug, or bone marrow suppression
200
Medication-Induced Thrombocytopenia: Etiologies
Penicillins, alcohol, chemotherapy, immunosuppressants
201
Bernard-Soulier Syndrome: Signs/Dx Criteria
Platelet-collagen adhesion defect
202
Bernard-Soulier Syndrome: Type
Bleeding Disorders
203
Bernard-Soulier Syndrome: Pathophysiology
No Gp1b receptor
204
Bernard-Soulier Syndrome: Etiologies
Rare inherited
205
Glanzmann Thrombocytopenia: Signs/Dx Criteria
Platelet-platelet adhesion defect
206
Glanzmann Thrombocytopenia: Type
Bleeding Disorders
207
Glanzmann Thrombocytopenia: Pathophysiology
GpIIb/IIIa defect
208
Glanzmann Thrombocytopenia: Etiologies
Rare inherited
209
Grey Platelet Syndrome: Type
Bleeding Disorders
210
Grey Platelet Syndrome: Pathophysiology
PLT granule defect
211
Grey Platelet Syndrome: Etiologies
Rare inherited
212
Heterozygous Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria
Increased venous thrombosis, some arterial thrombosis
213
Heterozygous Protein Deficiency: Type
Genetic Clotting Dzs
214
Homozygous Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria
Neonatal purpura fulminans, fibrinogenolysis, chronic DIC
215
Homozygous Protein Deficiency: Type
Genetic Clotting Dzs
216
Anticoagulant Protein Deficiency: Epi AND Symptoms AND Signs/Dx Criteria
Dominant: ↑VTE, (+)FHx. Recessive: no Hx or FHx of thrombosis
217
Anticoagulant Protein Deficiency: Type
Genetic Clotting Dzs
218
Anticoagulant Protein Deficiency: Pathophysiology AND Etiologies AND Treatment
↑thrombin generation, can be dominant or recessive inheritance
219
Factor V Leiden (aPC Resistance): Epi AND Symptoms AND Signs/Dx Criteria
↑VTE, synergistic effect in combo w/ other deficiencies
220
Factor V Leiden (aPC Resistance): Type
Genetic Clotting Dzs
221
Factor V Leiden (aPC Resistance): Pathophysiology AND Etiologies AND Treatment
Arg506Gln → Activated protein C fails to prolong aPTT
222
Prothrombin G20210→A: Epi
N. Europe
223
Prothrombin G20210→A: Symptoms AND Signs/Dx Criteria
↑prothrombin, thrombosis in pregnancy, VTE
224
Prothrombin G20210→A: Type
Genetic Clotting Dzs
225
Prothrombin G20210→A: Pathophysiology AND Etiologies AND Treatment
Mutation in non-coding sequence of prothrombin gene
226
Chronic Inflammatory Disease: Type
Acquired Clotting Disorders
227
Chronic Inflammatory Disease: Pathophysiology AND Etiologies
C4b = acute phase reactant, ↓protein S, ↓prothrombin-tenase breakdown; IL1 and TNF ↓thrombomodulin, thrombin→procoagulant
228
Chronic Inflammatory Disease: Treatment
Treat underlying cause
229
Nephrotic Syndrome: Signs/Dx Criteria
↑C4b, ↓S
230
Nephrotic Syndrome: Type
Acquired Clotting Disorders
231
Nephrotic Syndrome: Pathophysiology AND Etiologies
Lose proteins in urine (ATIII, S, C)
232
Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Symptoms
Spontaneous abortions
233
Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Signs/Dx Criteria
Venous and arterial thrombosis, false (+) RPR (syphilis), ↑aPTT in vitro only bc binds phospholipids
234
Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Type
Acquired Clotting Disorders
235
Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Pathophysiology
Antiphospholipid Abs
236
Antiphospholipid Antibody Syndrome (Lupus Anticoagulant): Etiologies
2° to vascular damage from autoimmune?
237
Thrombotic Thrombocytopenic Purpura: Symptoms
Bleeding and clotting!
238
Thrombotic Thrombocytopenic Purpura: Signs/Dx Criteria
Microangiopathic hemolytic anemia, thrombocytopenia, no coag abnormalities
239
Thrombotic Thrombocytopenic Purpura: Type
Acquired Clotting Disorders
240
Thrombotic Thrombocytopenic Purpura: Pathophysiology
↓ADAMTS13 (MMP for vWF)
241
Thrombotic Thrombocytopenic Purpura: Etiologies
Congenital (missing ADAMTS13) and/or Ab vs.
242
Thrombotic Thrombocytopenic Purpura: Treatment
Plasmapheresis / plasma exchange, steroids, rituximab, splenectomy
243
Heparin-Induced Thrombocytopenia (HIT): Signs/Dx Criteria
Thrombocytopenia 5-14 days s/p heparin, +/- thrombosis
244
Heparin-Induced Thrombocytopenia (HIT): Type
Acquired Clotting Disorders
245
Heparin-Induced Thrombocytopenia (HIT): Pathophysiology
Ig-mediated allergy to heparin
246
Heparin-Induced Thrombocytopenia (HIT): Etiologies
UFH > LMWH
247
Heparin-Induced Thrombocytopenia (HIT): Treatment
D/c heparin
248
Disseminated Intravascular Coagulation (DIC): Symptoms
Bleeding and clotting!
249
Disseminated Intravascular Coagulation (DIC): Signs/Dx Criteria
Hat trick: ↑PT, ↑PTT, ↑D-dimer. ↓PLT, +/- schistocytes, helmet cells
250
Disseminated Intravascular Coagulation (DIC): Type
Acquired Clotting Disorders
251
Disseminated Intravascular Coagulation (DIC): Pathophysiology
Consumptive disease, depletion of clotting factors
252
Disseminated Intravascular Coagulation (DIC): Etiologies
Hypertransfusion, disseminated CA, leukemia, infections
253
Disseminated Intravascular Coagulation (DIC): Treatment
Treat underlying cause; cryoprecipitate/FFP if fibrinogen <100, heparin
