Heme Flashcards

1
Q

Congenital aplastic anemia

A

Fanconi anemia, short stature, skin defects, hypogonadism, microcephaly, urogenital abnormalities

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2
Q

Work up for aplastic anemia

A

bone marrow, cytogenic analysis, B12, folate, LFTs, HIV, hepatitis, paroxysmal nocturnal hemoglobinuria screening

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3
Q

Treatment for aplastic anemia

A

allogenic stem cell transplant, or antithymocyte globulin and cyclosporine.

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4
Q

Cause of pure red cell aplasia

A

parvovirus b19, thymoma, autoimmune disorders, drugs

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5
Q

Large granular lymphocytosis

A

a common cause of pure red cell aplasia

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6
Q

Treatment for pure red cell aplasia

A

treat lymphoproliferative disorder, stop offending drugs, thymoma, prednisone, antithymocyte globulin, cyclosporine, cyclophosphamide, immune globulin if b19

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7
Q

Mild congenital neutropenia

A

middle eastern, African and black populations, not associated with increased infections, does not need treatment

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8
Q

Causes of neutropenia

A

drugs, vitamin deficiencies, autoimmune disorders, viral syndromes

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9
Q

Signs and symptoms of myelodysplastic syndromes

A

fatigue, easy bleeding, infections, anemia, nucleated erythrocytes, hypolobated hypogransular neutrophils

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10
Q

Treatment for myelodysplastic syndromes

A

observe if low risk, transfusions, iron chelation, epoetin, g-csf

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11
Q

Signs and symptoms of polycythemia vera

A

confusion, TIA, tinnitus, blurred vision, headache, pruritis, erythromelalgia (burning palms and soles), fever, weight loss, sweats, hepatosplenomegaly, DVT, bleeding, Budd-Chiari

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12
Q

Labs for polycythemia vera

A

HGB >18.5 in men, >16.5 women, leukocytosis, thrombocytosis, hepatosplenomegaly, elevated B12, uric acid, low erythropoietin, normal O2sat, JAK2 mutation

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13
Q

Treatment for polycythemia vera

A

low dose aspirin, phlebotomy and hydroxyurea (if over 60 or previous thrombosis)

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14
Q

Signs and symptoms of essential thrombocythemia

A

digital ischemia, erythromelalgia, bleeding, thrombosis, splenomegaly

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15
Q

Diagnosis of essential thrombocythemia

A

platelets >600,000 on 2 labs 1 month apart, no Philadelphia chromosome

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16
Q

Treatment for essential thrombocythemia

A

hydroxyurea plus low dose aspirin if thrombosis or >65 and risk for thrombosis

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17
Q

Signs and symptoms of CML

A

fatigue, night sweats, weight loss, abdominal discomfort, early satiety, bleeding, splenomegaly

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18
Q

Diagnosis of CML

A

neutrophilia, left shift, basophilia, elevated LDH, uric acid, low LAP, Philadelphia chromosome

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19
Q

Treatment for CML

A

imatinib

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20
Q

Symptoms of primary myelofibrosis

A

fatigue, night sweats, weight loss, abdominal pain, early satiety

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21
Q

Signs of primary myelofibrosis

A

hepatosplenomegaly, gout, bone pain, thrombosis, pulmonary hypertension

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22
Q

Diagnosis of primary myelofibrosis

A

anemia, left shift , nucleated and teardrop erythrocytes, elevated LDH, uric acid, alkaline phosphatase

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23
Q

Treatment for primary myelofibrosis

A

observation if asymptomatic. if symptomatic, then transfusions, danazol, hydroxyurea, splenectomy, stem cell transplant

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24
Q

Risk factors for acute myeloid leukemia

A

DNA damage, MDS or myeloproliferative disorder, defect in DNA repair, Down syndrome, aplastic anemia or paroxysmal nocturnal hemoglobinuria

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25
Q

Clinical manifestations of acute myeloid leukemia

A

fatigue, dyspnea, easy bleeding, fever, gingival hypertrophy, violaceous nontender cutaneous plaques, acute neutrophilic dermatosis (Sweet syndrome)

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26
Q

Diagnosis of acute myeloid leukemia

A

20% myeloblasts in blood or bone marrow, Auer rods, cytopenias, DIC, tumor lysis syndrome, hypokalemia

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27
Q

Treatment for acute myeloid leukemia

A

transfusions, allopurinol, chemo with cytarabine, and anthracycline

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28
Q

What is acute promyelocytic leukemia

A

variant of acute myeloid leukemia with t(15;17) sensitive to all trans retinoic acid

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29
Q

Clinical manifestations of acute lymphoblastic leukemia

A

fatigue, dyspnea, bleeding, fever, lymphadenopathy, hepatospenomegaly. anterior mediastinal mass, pleural effusion, superior vena cava syndrome. headaches, lethargy, nausea, vomiting, nuchal rigidity, cranial palsy, radiculopathy

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30
Q

Diagnosis of acute lymphoblastic leukemia

A

25% lymphoblasts in bone marrow

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31
Q

Treatment for acute lymphoblastic leukemia

A

allopurinol, chemo, intrathecal chemo, stem cell transplant. if Philadelphia chromosome positive then imatinib

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32
Q

Requirement for erythropoiesis stimulating agent

A

HGB 20%, ferritin >100

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33
Q

Clinical manifestations of multiple myeloma

A

bone pain, spinal cord compression, hyperviscosity, anemia, hypercalcemia, elevated creatinine

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34
Q

Diagnosis of symptomatic multiple myeloma

A

10% bone marrow plasma cells, an M protein present, and end organ damage.

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35
Q

Diagnosis of asymptomatic multiple myeloma

A

10% bone marrow plasma cells, 3g/dL M protein, and not end organ damage

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36
Q

Diagnosis of monoclonal gammopathy of undetermined significance

A

<10% bone marrow plasma cells, an M protein present, and no end organ damage

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37
Q

Clinical manifestations of AL amyloidosis

A

nephrotic range proteinuria with worsening kidney function, restrictive cardiomyopathy, hepatomegaly, symmetric distal sensorimotor neuropathy, carpal tunnel syndrome, autonomic neuropathy, Raccoon eyes, macroglossia

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38
Q

Diagnosis of AL amyloidosis

A

the presence of monoclonal plasma cell disorder, amyloid deposits of clonal light chains, and characteristic amyloid deposits (apple green birefringence with Congo red stain)

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39
Q

Waldenstrom macroglobulinemia

A

lymphoplasmacytic lymphoma characterized by the production of monoclonal IgM antibodies

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40
Q

Clinical manifestations of Waldenstrom macroglobulinemia

A

constitutional symptoms, lymphadenopathy, hepatosplenomegaly, hyperviscosity, peripheral sensorimotor neuropathy, mucosal bleeding, anemia with rouleaux formation

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41
Q

Signs and symptoms of hyperviscosity

A

headaches, blurred vision, dizziness, tinnitus, deafness, mental status changes, dilated tortuous retinal veins and retinal hemorrhage

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42
Q

Diagnosis of Waldenstrom macroglobulinemia

A

lymphoplasmacytic lymphoma of 10% of bone marrow and presence of IgM M protein

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43
Q

Treatment of Waldenstrom macroglobulinemia

A

Symptomatic patients only. Plasmapheresis of hyperviscosity, and Rituximab

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44
Q

Spherocytes

A

hereditary spherocytosis, warm autoimmune hemolytic anemia

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45
Q

Macrocytes

A

B12 of folate deficiency, myelodysplasia, antimetabolites

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46
Q

Target cells

A

hemoglobinopathy, liver disease, splenectomy

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47
Q

Schistocytes

A

microangiopathy (TTP, HUS, DIC)

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48
Q

Nucleated erythrocytes

A

marrow stress (hemolysis, hypoxia)

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49
Q

Teardrop cells

A

Fibrosis, marrow infiltration

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50
Q

Bite cells

A

G6PD deficiency

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51
Q

Rouleaux

A

paraproteinemia (myeloma)

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52
Q

Burr cells (gears)

A

kidney disease

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53
Q

Spur cells

A

severe liver disease

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54
Q

Elliptocytes

A

hereditary elliptocytosis

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55
Q

Hepcidin

A

produced by the liver to reduce excess iron absorption

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56
Q

Koilonychia

A

spooning of the nails due to severe iron deficiency

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57
Q

Characteristics of B12 deficiency

A

glossitis, weight loss, pale yellow skin, loss of position sense and vibration sense, hallucinations, dementia, psychosis

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58
Q

Peripheral smear in B12 deficiency

A

oval macrocytes with basophilic stippling, hypersegmented neutrophils with more than 5 lobes

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59
Q

Diagnose low-normal range B12 deficiency

A

elevated methylmalonic acid and homocysteine

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60
Q

Drugs that cause folate deficiency

A

triamterine, phenytoin, alcohol

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61
Q

Diagnose normal range folate deficiency

A

elevated homocysteine, but not methylmalonic acid

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62
Q

Inflammatory anemia

A

inflammatory cytokines cause low erythropoietin levels, and increased hepcidin

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63
Q

Anemia of kidney disease

A

low erythropoietin levels

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64
Q

Guidelines for erythropoietin use

A

target hemoglobin levels 11-12 g/dL, ferritin >100, iron saturation > 20%

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65
Q

Labs supporting hemolytic anemia

A

increased reticulocyte count, indirect bilirubin, and LDH levels, and decreased haptoglobin

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66
Q

Supporting history of hereditary spherocytosis

A

personal or family history of anemia, jaundice, splenomegaly, gallstones

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67
Q

Tests for hereditary spherocytosis

A

Coombs test is negative. osmotic fragility test is postive

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68
Q

Trigger of G6PD deficiency hemolysis

A

oxidative stressors such as infection, drugs such as dapsone, bactrim, nitrofurantoin, and fava beans

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69
Q

Heinz body

A

blue stained oxidized hemoglobin body in G6PD deficiency anemia, seen with bite cells

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70
Q

Treatment for hereditary spherocytosis

A

supportive, and splenectomy

71
Q

Treatment for G6PD deficiency anemia

A

supportive, remove offending drug

72
Q

Alpha Thalassemia minor

A

mild anemia, microcytosis, hypochromia, target cells, normal hemoglobin electrophoresis

73
Q

RDW for thalassemia

A

RDW is elevated in iron deficiency anemia, but normal in thalassemia

74
Q

Hemoglobin H

A

severe alpha thalassemia with severe anemia, heart failure, hypoxia, identifiable on hemoglobin electrophoresis

75
Q

Hydrops fetalis

A

severe alpha thalassemia typically associated with utero fetal demise

76
Q

Beta Thalassemia trait

A

mild anemia, microcytosis, hypochromia, target cells, increased hemoglobin A2 and F on electrophoresis

77
Q

Beta Thalassemia major

A

severe anemia, growth retardation, skeletal complications and iron overload

78
Q

Treatment for Beta Thalassemia major

A

lifelong transfusions, iron chelation, splenectomy, stem cell transplant

79
Q

Sickle cell Hemoglobin SS

A

reduced life expectancy, moderate to severe anemia, frequent pain crises

80
Q

Treatment for Sickle cell disease

A

hydroxyurea, hydration, analgesics, incentive spirometry

81
Q

Treatment for stroke in Sickle cell disease

A

exchange transfusion to reduce Hb S to <30%

82
Q

Treatment of acute chest syndrome in Sickle cell disease

A

broad spectrum antibiotics, oxygen, analgesics, avoid overhydration, and possibly bronchodilators and exchange transfusion

83
Q

Diagnose warm autoimmune hemolytic anemia

A

splenomegaly, jaundice, spherocytes, direct Coombs test positive for IgG and negative or weakly positive for C3

84
Q

Treatment for warm autoimmune hemolytic anemia

A

steroids

85
Q

Warm autoimmune hemolytic anemia

A

IgG binds erythrocytes, cleared by spleen

86
Q

Cold agglutinin disease

A

IgM binds erythrocytes, fix complement, cleared by direct intravascular lysis and liver

87
Q

Diagnosis of cold agglutinin disease

A

clumping of erythrocytes, direct Coombs negative for IgG, and positive for C3

88
Q

Treatment for cold agglutinin disease

A

avoid cold

89
Q

Cause of drug induced autoimmune hemolytic anemia

A

many drugs, especially 2-3rd generation cephalosporins

90
Q

Cause of microangiopathic hemolytic anemia

A

lesions that expose erythrocytes to high shear stress such as mechanical heart valve, also TTP, HUS, DIC, HELLP

91
Q

Diagnosis of microangiopathic hemolytic anemia

A

schistocytes or helmet cells, low haptoglobin level, hemoglobinuria, and elevated serum LDH

92
Q

Treatment for microangiopathic hemolytic anemia

A

plasma exchange therapy for TTP, HUS

93
Q

Paroxysmal nocturnal hemoglobinuria

A

clonal stem cell disorder causing hemolytic anemia, pancytopenia and atypical thrombosis

94
Q

Treatment for paroxysmal nocturnal hemoglobinuria

A

anticoagulation if thrombosis or >50% CD55 or CD59 deficient

95
Q

Infectious causes of hemolytic anemia

A

malaria, babesiosis

96
Q

Clinical manifestations of hemochromatosis

A

cardiomyopathy, arrhythmia, cirrhosis, diabetes, arthropathy, skin bronzing

97
Q

Diagnosis of hemochromatosis

A

transferrin saturation >60% in men, >50% in women

98
Q

Treatment for hemochromatosis

A

phlebotomy to ferritin <50

99
Q

Hemoglobin transfusion volume

A

250mL yielding increase in hemoglobin of 1 g/dL

100
Q

Hemoglobin transfusion target

A

critically ill patients not actively bleeding and with no cardiac compromise at 7 g/dL

101
Q

Platelet transfusion volume increase

A

20,000-30,000 /uL

102
Q

Platelet transfusion target

A

For non CNS surgical or active bleeding 50,000-100,000. For CNS bleeding or planned CNS surgery then >100,000

103
Q

Indication for Fresh frozen plasma

A

warfarin reversal in actively bleeding patients, TTP, dilutional coagulopathy, and bleeding patients with factor deficiencies

104
Q

Indication for Cryoprecipitate

A

active bleeding with hypofibrinogenemia from liver disease, thrombotic therapy, or DIC

105
Q

Cause of acute hemolytic transfusion reaction

A

ABO incompatibility

106
Q

Effects of acute hemolytic transfusion reaction

A

hypotension, fever, kidney failure, pain at infusion site, and DIC

107
Q

Treatment for acute hemolytic transfusion reaction

A

fluid resuscitation, vasopressor support, and mannitol

108
Q

Cause of delayed hemolytic transfusion reaction

A

amnestic response to reexposure to a non-ABO alloantibody

109
Q

Effects of delayed hemolytic transfusion reaction

A

5-10 days later, anemia, jaundice and fever

110
Q

Signs of transfusion associated circulatory overload

A

dyspnea, cough, tachycardia, cyanosis, edema, chest tightness, caused by fluid overload

111
Q

Cause of transfusion related acute lung injury

A

antibodies in donor plasma directed against recipient neutrophil antigens, causing sequestration in the lung, and capillary leak

112
Q

Signs of transfusion related acute lung injury

A

hypoxia, dyspnea, fever, hypotension, bilateral infiltrates with pulmonary edema, within 6 hrs of transfusion

113
Q

Cause of febrile nonhemolytic transfusion reaction

A

recipient derived leukoreactive antibodies and donor derived cytokines

114
Q

Treatment for febrile nonhemolytic transfusion reaction

A

stop transfusion until acute hemolytic transfusion reaction can be excluded. pretransfusion antipyretics and leukoreduction

115
Q

Cause of transfusion anaphylaxis reaction

A

IgA deficient patients have antibodies to IgA, that react to IgA contained in the transfused plasma

116
Q

Who is at risk for transfusion associated graft verses host disease

A

stem cell transplant recipients, recipients of transfusion from first degree relatives, and patients with immunosuppression associated with hematologic malignancies such as Hodgkin lymphoma

117
Q

Prevention of transfusion associated graft verses host diease

A

irradiation of cellular products before transfustion

118
Q

Bleeding history

A

bleeding into muscle or joint indicates a disorder of humoral clotting factors, whereas mucosal bleeding indicates disorder of primary hemostasis, thrombocytopenia, or qualitative platelet disorders

119
Q

Prolonged PT, normal PTT

A

acquired deficiency of factor VII, vitamin K deficiency, liver disease, DIC, or warfarin

120
Q

Prolonged PTT, normal PT

A

lupus inhibitor, hemophilia

121
Q

Prolonged thrombin clotting time

A

thrombin inhibitor such as heparin, decreased or abnormal fibrin such as dysfibrinogenemia

122
Q

Mixing study

A

correction of the clotting time indicates a factor deficiency, whereas incomplete correction indicates a factor inhibitor

123
Q

Hemophilia A and B

A

X linked recessive deficiency of factor VIII and IX respectively

124
Q

Clinical manifestation or hemophilia

A

recurrent hemarthoses that result in chronic, crippling degenerative joint disease and CNS hemorrhage

125
Q

von Willebrand disease

A

vWF protects factor VIII from degradation

126
Q

Clinical manifestation of von Willebrand disease

A

mucosal bleeding, menorrhagia, postpartum hemorrhage

127
Q

Treatment for von Willebrand disease

A

infusion of vWF containing factor VIII concentrates

128
Q

Cause of acquired hemophilia

A

postpartum state, malignancy, autoimmune disease, idiopathic

129
Q

Treatment for acquired hemophilia

A

if low titer of inhibitor, then factor VIII concentrates. otherwise recombinant factor VIIa concentrate or prothrombin complex

130
Q

Treatment for coagulopathy of liver disease

A

fresh frozen plasma and vitamin K

131
Q

Disseminated intravascular coagulation

A

abnormal thrombin generation, rapid consumption of clotting factors and platelets, and accelerated fibrinolysis.

132
Q

Diagnosis of DIC

A

low platelet levels, prolonged PT, low or decreasing fibrinogen level, elevated D-dimer, schistocytes

133
Q

Treatment of DIC

A

treat underlying cause, transfusions of plasma, cryoprecipitate, and platelets

134
Q

Cryoprecipitate

A

concentrated source of factor VIII, vWF, factor XIII, and fibrinogen.

135
Q

Vitamin K

A

required for generation of the active form of factors, II, VII, IX, X

136
Q

Immune thrombocytopenic purpura

A

acquired autoimmune disease with autoantibodies directed against platelet surface proteins

137
Q

Diagnose immune thrombocytopenic purpura

A

mild to severe bleeding, otherwise normal blood counts, large (new) platelets

138
Q

Treatment for immune thrombocytopenic purpura

A

steroids, IVIG, rituximab, splenectomy

139
Q

Causes of immune thrombocytopenic purpura

A

Beta-lactams, cephalosporins, sulfa containing drugs, quinine, vancomycin, G IIb/IIIa inhibitors

140
Q

Diagnose heparin induced thrombocytopenia

A

develops 5-10 days after heparin exposure, platelet count decrease >50%, often with thrombotic event

141
Q

Treatment for heparin induced thrombocytopenia

A

stop heparin, anticoagulate with lepirudin, argatroban, danaparoid, or bivalirudin

142
Q

Thrombotic thrombocytopenic purpura

A

abnormal activation of platelets and endothelial cells, deposition of fibrin in the microvasculature, and peripheral destruction of erythrocytes and platelets

143
Q

Signs of TTP

A

schistocytes, elevated serum LDH, thrombocytopenia. fever, hematuria, elevated creatinine, proteinuria, headache, confusion, seizures, stroke.

144
Q

Treatment for TTP

A

plasma exchange

145
Q

Hemolytic uremic syndrome

A

similar to TTP, caused by toxin from Ecoli or Shigella causing renal cell death

146
Q

Treatment for HUS

A

plasma exchange

147
Q

Treatment for uremia and active bleeding

A

desmopression, dialysis, conjugated estrogens

148
Q

Factor V Leiden

A

mutation disrupts cleavage site on activated protein C, slowing the degradation of factor Va and factor VIIIa, increasing risk of venous thrombosis

149
Q

Prothrombin G20210A mutation

A

mutation stabilizes prothrombin mRNA, increasing prothrombin levels, increasing risk of venous thrombosis

150
Q

Antithrombin deficiency

A

mutation affects antithrombin protein that inhibits serine proteases such as thrombin and factor Xa, resulting in increased risk for thromboembolism

151
Q

Protein C deficiency

A

mutation affects protein C, a serine protease that inactivates factors Va and VIIIa, resulting in increased risk for thromboembolism

152
Q

Protein S deficiency

A

mutation affects protein S, a cofactor for protein C, thus impairs degradation of factors Va and VIIIa. also impairs degradation of factor Xa by tissue factor pathway. results in increased risk for thromboembolism.

153
Q

Acquired thrombophilic conditions

A

surgery, trauma, cancer, antiphospholipid syndrome

154
Q

Antiphospholipid syndrome

A

acquired autoimmune disorder that induces tissue factor expression, disrupts protein C and antithrombin function, and activates platelets and the complement cascade

155
Q

Function of heparin

A

binds to antithrombin and accelerates the inhibition of activated serine proteases factor Xa and thrombin

156
Q

How is heparin cleared

A

UFH by endothelial cells and macrophages, while LMWH is renally cleared

157
Q

Treatment for ductal carcinoma in situ breast cancer

A

lumpectomy or mastectomy with tamoxifen if estrogen receptor postive

158
Q

Treatment for invasive breast cancer

A

lumpectomy or mastectomy, with sentinel node biopsy, followed by whole breast radiation

159
Q

High risk for ovarian cancer

A

positive for BRCA or HNPCC should have prophylactic bilateral salpingo-oophorectomy before age 40

160
Q

Signs of ovarian cancer

A

postmenopausal women with abdominal distention, pain, nausea, bloating, anorexia, ascites

161
Q

Monitoring after ovarian cancer treatment

A

CA125

162
Q

Testicular cancer tumor markers

A

elevated alpha fetoprotein defines a non-seminoma. beta HCG in both seminoma and non-seminoma, and serum LDH indicates rapid cell turnover.

163
Q

Who gets non-Hodgkin lymphoma

A

men more than women, older age, more common in those who are immunosuppressed

164
Q

Who gets Hodgkin lymphoma

A

age 15-45 and >55

165
Q

Biopsy required for diagnosis of lymphoma

A

excisional biopsy rather than fine needle aspiration

166
Q

Follicular lymphoma

A

median age 60, men and women equally, delay treatment until symptomatic. treat with rituximab +- CVP or CHOP

167
Q

MALT lymphoma

A

associated with H pylori, remission with treatment of infection

168
Q

Chronic lymphocytic leukemia

A

median age 70, delay treatment until symptomatic, treat with rituximab + fludarabine

169
Q

Hairy cell leukemia

A

older adults, men more commonly, with progressive cytopenia and splenomegaly without lymphadenopathy. treat with cladribine

170
Q

Diffuse large cell lymphoma

A

B and T cell types. B cell lymphoma with lymphadenopathy and fever, night sweats and weight loss

171
Q

Superior vena cava syndrome

A

most commonly caused by lung cancer, causes dyspnea, facial swelling, cough, distension of neck veins, facial edema, cyanosis, facial plethora

172
Q

Tumor lysis syndrome

A

lysis of tumor cells causes hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, DIC and acute kidney failure

173
Q

Malignancy associated hypercalcemia

A

nausea, vomiting, constipation, polyuria, polydipsia, weakness, confusion