Heme Flashcards
1
Q
Congenital aplastic anemia
A
Fanconi anemia, short stature, skin defects, hypogonadism, microcephaly, urogenital abnormalities
2
Q
Work up for aplastic anemia
A
bone marrow, cytogenic analysis, B12, folate, LFTs, HIV, hepatitis, paroxysmal nocturnal hemoglobinuria screening
3
Q
Treatment for aplastic anemia
A
allogenic stem cell transplant, or antithymocyte globulin and cyclosporine.
4
Q
Cause of pure red cell aplasia
A
parvovirus b19, thymoma, autoimmune disorders, drugs
5
Q
Large granular lymphocytosis
A
a common cause of pure red cell aplasia
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
7
Q
Mild congenital neutropenia
A
middle eastern, African and black populations, not associated with increased infections, does not need treatment
8
Q
Causes of neutropenia
A
drugs, vitamin deficiencies, autoimmune disorders, viral syndromes
9
Q
Signs and symptoms of myelodysplastic syndromes
A
fatigue, easy bleeding, infections, anemia, nucleated erythrocytes, hypolobated hypogransular neutrophils
10
Q
Treatment for myelodysplastic syndromes
A
observe if low risk, transfusions, iron chelation, epoetin, g-csf
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
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
13
Q
Treatment for polycythemia vera
A
low dose aspirin, phlebotomy and hydroxyurea (if over 60 or previous thrombosis)
14
Q
Signs and symptoms of essential thrombocythemia
A
digital ischemia, erythromelalgia, bleeding, thrombosis, splenomegaly
15
Q
Diagnosis of essential thrombocythemia
A
platelets >600,000 on 2 labs 1 month apart, no Philadelphia chromosome
16
Q
Treatment for essential thrombocythemia
A
hydroxyurea plus low dose aspirin if thrombosis or >65 and risk for thrombosis
17
Q
Signs and symptoms of CML
A
fatigue, night sweats, weight loss, abdominal discomfort, early satiety, bleeding, splenomegaly
18
Q
Diagnosis of CML
A
neutrophilia, left shift, basophilia, elevated LDH, uric acid, low LAP, Philadelphia chromosome
19
Q
Treatment for CML
A
imatinib
20
Q
Symptoms of primary myelofibrosis
A
fatigue, night sweats, weight loss, abdominal pain, early satiety
21
Q
Signs of primary myelofibrosis
A
hepatosplenomegaly, gout, bone pain, thrombosis, pulmonary hypertension
22
Q
Diagnosis of primary myelofibrosis
A
anemia, left shift , nucleated and teardrop erythrocytes, elevated LDH, uric acid, alkaline phosphatase
23
Q
Treatment for primary myelofibrosis
A
observation if asymptomatic. if symptomatic, then transfusions, danazol, hydroxyurea, splenectomy, stem cell transplant
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
25
Clinical manifestations of acute myeloid leukemia
fatigue, dyspnea, easy bleeding, fever, gingival hypertrophy, violaceous nontender cutaneous plaques, acute neutrophilic dermatosis (Sweet syndrome)
26
Diagnosis of acute myeloid leukemia
20% myeloblasts in blood or bone marrow, Auer rods, cytopenias, DIC, tumor lysis syndrome, hypokalemia
27
Treatment for acute myeloid leukemia
transfusions, allopurinol, chemo with cytarabine, and anthracycline
28
What is acute promyelocytic leukemia
variant of acute myeloid leukemia with t(15;17) sensitive to all trans retinoic acid
29
Clinical manifestations of acute lymphoblastic leukemia
fatigue, dyspnea, bleeding, fever, lymphadenopathy, hepatospenomegaly. anterior mediastinal mass, pleural effusion, superior vena cava syndrome. headaches, lethargy, nausea, vomiting, nuchal rigidity, cranial palsy, radiculopathy
30
Diagnosis of acute lymphoblastic leukemia
25% lymphoblasts in bone marrow
31
Treatment for acute lymphoblastic leukemia
allopurinol, chemo, intrathecal chemo, stem cell transplant. if Philadelphia chromosome positive then imatinib
32
Requirement for erythropoiesis stimulating agent
HGB 20%, ferritin >100
33
Clinical manifestations of multiple myeloma
bone pain, spinal cord compression, hyperviscosity, anemia, hypercalcemia, elevated creatinine
34
Diagnosis of symptomatic multiple myeloma
10% bone marrow plasma cells, an M protein present, and end organ damage.
35
Diagnosis of asymptomatic multiple myeloma
10% bone marrow plasma cells, 3g/dL M protein, and not end organ damage
36
Diagnosis of monoclonal gammopathy of undetermined significance
<10% bone marrow plasma cells, an M protein present, and no end organ damage
37
Clinical manifestations of AL amyloidosis
nephrotic range proteinuria with worsening kidney function, restrictive cardiomyopathy, hepatomegaly, symmetric distal sensorimotor neuropathy, carpal tunnel syndrome, autonomic neuropathy, Raccoon eyes, macroglossia
38
Diagnosis of AL amyloidosis
the presence of monoclonal plasma cell disorder, amyloid deposits of clonal light chains, and characteristic amyloid deposits (apple green birefringence with Congo red stain)
39
Waldenstrom macroglobulinemia
lymphoplasmacytic lymphoma characterized by the production of monoclonal IgM antibodies
40
Clinical manifestations of Waldenstrom macroglobulinemia
constitutional symptoms, lymphadenopathy, hepatosplenomegaly, hyperviscosity, peripheral sensorimotor neuropathy, mucosal bleeding, anemia with rouleaux formation
41
Signs and symptoms of hyperviscosity
headaches, blurred vision, dizziness, tinnitus, deafness, mental status changes, dilated tortuous retinal veins and retinal hemorrhage
42
Diagnosis of Waldenstrom macroglobulinemia
lymphoplasmacytic lymphoma of 10% of bone marrow and presence of IgM M protein
43
Treatment of Waldenstrom macroglobulinemia
Symptomatic patients only. Plasmapheresis of hyperviscosity, and Rituximab
44
Spherocytes
hereditary spherocytosis, warm autoimmune hemolytic anemia
45
Macrocytes
B12 of folate deficiency, myelodysplasia, antimetabolites
46
Target cells
hemoglobinopathy, liver disease, splenectomy
47
Schistocytes
microangiopathy (TTP, HUS, DIC)
48
Nucleated erythrocytes
marrow stress (hemolysis, hypoxia)
49
Teardrop cells
Fibrosis, marrow infiltration
50
Bite cells
G6PD deficiency
51
Rouleaux
paraproteinemia (myeloma)
52
Burr cells (gears)
kidney disease
53
Spur cells
severe liver disease
54
Elliptocytes
hereditary elliptocytosis
55
Hepcidin
produced by the liver to reduce excess iron absorption
56
Koilonychia
spooning of the nails due to severe iron deficiency
57
Characteristics of B12 deficiency
glossitis, weight loss, pale yellow skin, loss of position sense and vibration sense, hallucinations, dementia, psychosis
58
Peripheral smear in B12 deficiency
oval macrocytes with basophilic stippling, hypersegmented neutrophils with more than 5 lobes
59
Diagnose low-normal range B12 deficiency
elevated methylmalonic acid and homocysteine
60
Drugs that cause folate deficiency
triamterine, phenytoin, alcohol
61
Diagnose normal range folate deficiency
elevated homocysteine, but not methylmalonic acid
62
Inflammatory anemia
inflammatory cytokines cause low erythropoietin levels, and increased hepcidin
63
Anemia of kidney disease
low erythropoietin levels
64
Guidelines for erythropoietin use
target hemoglobin levels 11-12 g/dL, ferritin >100, iron saturation > 20%
65
Labs supporting hemolytic anemia
increased reticulocyte count, indirect bilirubin, and LDH levels, and decreased haptoglobin
66
Supporting history of hereditary spherocytosis
personal or family history of anemia, jaundice, splenomegaly, gallstones
67
Tests for hereditary spherocytosis
Coombs test is negative. osmotic fragility test is postive
68
Trigger of G6PD deficiency hemolysis
oxidative stressors such as infection, drugs such as dapsone, bactrim, nitrofurantoin, and fava beans
69
Heinz body
blue stained oxidized hemoglobin body in G6PD deficiency anemia, seen with bite cells
70
Treatment for hereditary spherocytosis
supportive, and splenectomy
71
Treatment for G6PD deficiency anemia
supportive, remove offending drug
72
Alpha Thalassemia minor
mild anemia, microcytosis, hypochromia, target cells, normal hemoglobin electrophoresis
73
RDW for thalassemia
RDW is elevated in iron deficiency anemia, but normal in thalassemia
74
Hemoglobin H
severe alpha thalassemia with severe anemia, heart failure, hypoxia, identifiable on hemoglobin electrophoresis
75
Hydrops fetalis
severe alpha thalassemia typically associated with utero fetal demise
76
Beta Thalassemia trait
mild anemia, microcytosis, hypochromia, target cells, increased hemoglobin A2 and F on electrophoresis
77
Beta Thalassemia major
severe anemia, growth retardation, skeletal complications and iron overload
78
Treatment for Beta Thalassemia major
lifelong transfusions, iron chelation, splenectomy, stem cell transplant
79
Sickle cell Hemoglobin SS
reduced life expectancy, moderate to severe anemia, frequent pain crises
80
Treatment for Sickle cell disease
hydroxyurea, hydration, analgesics, incentive spirometry
81
Treatment for stroke in Sickle cell disease
exchange transfusion to reduce Hb S to <30%
82
Treatment of acute chest syndrome in Sickle cell disease
broad spectrum antibiotics, oxygen, analgesics, avoid overhydration, and possibly bronchodilators and exchange transfusion
83
Diagnose warm autoimmune hemolytic anemia
splenomegaly, jaundice, spherocytes, direct Coombs test positive for IgG and negative or weakly positive for C3
84
Treatment for warm autoimmune hemolytic anemia
steroids
85
Warm autoimmune hemolytic anemia
IgG binds erythrocytes, cleared by spleen
86
Cold agglutinin disease
IgM binds erythrocytes, fix complement, cleared by direct intravascular lysis and liver
87
Diagnosis of cold agglutinin disease
clumping of erythrocytes, direct Coombs negative for IgG, and positive for C3
88
Treatment for cold agglutinin disease
avoid cold
89
Cause of drug induced autoimmune hemolytic anemia
many drugs, especially 2-3rd generation cephalosporins
90
Cause of microangiopathic hemolytic anemia
lesions that expose erythrocytes to high shear stress such as mechanical heart valve, also TTP, HUS, DIC, HELLP
91
Diagnosis of microangiopathic hemolytic anemia
schistocytes or helmet cells, low haptoglobin level, hemoglobinuria, and elevated serum LDH
92
Treatment for microangiopathic hemolytic anemia
plasma exchange therapy for TTP, HUS
93
Paroxysmal nocturnal hemoglobinuria
clonal stem cell disorder causing hemolytic anemia, pancytopenia and atypical thrombosis
94
Treatment for paroxysmal nocturnal hemoglobinuria
anticoagulation if thrombosis or >50% CD55 or CD59 deficient
95
Infectious causes of hemolytic anemia
malaria, babesiosis
96
Clinical manifestations of hemochromatosis
cardiomyopathy, arrhythmia, cirrhosis, diabetes, arthropathy, skin bronzing
97
Diagnosis of hemochromatosis
transferrin saturation >60% in men, >50% in women
98
Treatment for hemochromatosis
phlebotomy to ferritin <50
99
Hemoglobin transfusion volume
250mL yielding increase in hemoglobin of 1 g/dL
100
Hemoglobin transfusion target
critically ill patients not actively bleeding and with no cardiac compromise at 7 g/dL
101
Platelet transfusion volume increase
20,000-30,000 /uL
102
Platelet transfusion target
For non CNS surgical or active bleeding 50,000-100,000. For CNS bleeding or planned CNS surgery then >100,000
103
Indication for Fresh frozen plasma
warfarin reversal in actively bleeding patients, TTP, dilutional coagulopathy, and bleeding patients with factor deficiencies
104
Indication for Cryoprecipitate
active bleeding with hypofibrinogenemia from liver disease, thrombotic therapy, or DIC
105
Cause of acute hemolytic transfusion reaction
ABO incompatibility
106
Effects of acute hemolytic transfusion reaction
hypotension, fever, kidney failure, pain at infusion site, and DIC
107
Treatment for acute hemolytic transfusion reaction
fluid resuscitation, vasopressor support, and mannitol
108
Cause of delayed hemolytic transfusion reaction
amnestic response to reexposure to a non-ABO alloantibody
109
Effects of delayed hemolytic transfusion reaction
5-10 days later, anemia, jaundice and fever
110
Signs of transfusion associated circulatory overload
dyspnea, cough, tachycardia, cyanosis, edema, chest tightness, caused by fluid overload
111
Cause of transfusion related acute lung injury
antibodies in donor plasma directed against recipient neutrophil antigens, causing sequestration in the lung, and capillary leak
112
Signs of transfusion related acute lung injury
hypoxia, dyspnea, fever, hypotension, bilateral infiltrates with pulmonary edema, within 6 hrs of transfusion
113
Cause of febrile nonhemolytic transfusion reaction
recipient derived leukoreactive antibodies and donor derived cytokines
114
Treatment for febrile nonhemolytic transfusion reaction
stop transfusion until acute hemolytic transfusion reaction can be excluded. pretransfusion antipyretics and leukoreduction
115
Cause of transfusion anaphylaxis reaction
IgA deficient patients have antibodies to IgA, that react to IgA contained in the transfused plasma
116
Who is at risk for transfusion associated graft verses host disease
stem cell transplant recipients, recipients of transfusion from first degree relatives, and patients with immunosuppression associated with hematologic malignancies such as Hodgkin lymphoma
117
Prevention of transfusion associated graft verses host diease
irradiation of cellular products before transfustion
118
Bleeding history
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
Prolonged PT, normal PTT
acquired deficiency of factor VII, vitamin K deficiency, liver disease, DIC, or warfarin
120
Prolonged PTT, normal PT
lupus inhibitor, hemophilia
121
Prolonged thrombin clotting time
thrombin inhibitor such as heparin, decreased or abnormal fibrin such as dysfibrinogenemia
122
Mixing study
correction of the clotting time indicates a factor deficiency, whereas incomplete correction indicates a factor inhibitor
123
Hemophilia A and B
X linked recessive deficiency of factor VIII and IX respectively
124
Clinical manifestation or hemophilia
recurrent hemarthoses that result in chronic, crippling degenerative joint disease and CNS hemorrhage
125
von Willebrand disease
vWF protects factor VIII from degradation
126
Clinical manifestation of von Willebrand disease
mucosal bleeding, menorrhagia, postpartum hemorrhage
127
Treatment for von Willebrand disease
infusion of vWF containing factor VIII concentrates
128
Cause of acquired hemophilia
postpartum state, malignancy, autoimmune disease, idiopathic
129
Treatment for acquired hemophilia
if low titer of inhibitor, then factor VIII concentrates. otherwise recombinant factor VIIa concentrate or prothrombin complex
130
Treatment for coagulopathy of liver disease
fresh frozen plasma and vitamin K
131
Disseminated intravascular coagulation
abnormal thrombin generation, rapid consumption of clotting factors and platelets, and accelerated fibrinolysis.
132
Diagnosis of DIC
low platelet levels, prolonged PT, low or decreasing fibrinogen level, elevated D-dimer, schistocytes
133
Treatment of DIC
treat underlying cause, transfusions of plasma, cryoprecipitate, and platelets
134
Cryoprecipitate
concentrated source of factor VIII, vWF, factor XIII, and fibrinogen.
135
Vitamin K
required for generation of the active form of factors, II, VII, IX, X
136
Immune thrombocytopenic purpura
acquired autoimmune disease with autoantibodies directed against platelet surface proteins
137
Diagnose immune thrombocytopenic purpura
mild to severe bleeding, otherwise normal blood counts, large (new) platelets
138
Treatment for immune thrombocytopenic purpura
steroids, IVIG, rituximab, splenectomy
139
Causes of immune thrombocytopenic purpura
Beta-lactams, cephalosporins, sulfa containing drugs, quinine, vancomycin, G IIb/IIIa inhibitors
140
Diagnose heparin induced thrombocytopenia
develops 5-10 days after heparin exposure, platelet count decrease >50%, often with thrombotic event
141
Treatment for heparin induced thrombocytopenia
stop heparin, anticoagulate with lepirudin, argatroban, danaparoid, or bivalirudin
142
Thrombotic thrombocytopenic purpura
abnormal activation of platelets and endothelial cells, deposition of fibrin in the microvasculature, and peripheral destruction of erythrocytes and platelets
143
Signs of TTP
schistocytes, elevated serum LDH, thrombocytopenia. fever, hematuria, elevated creatinine, proteinuria, headache, confusion, seizures, stroke.
144
Treatment for TTP
plasma exchange
145
Hemolytic uremic syndrome
similar to TTP, caused by toxin from Ecoli or Shigella causing renal cell death
146
Treatment for HUS
plasma exchange
147
Treatment for uremia and active bleeding
desmopression, dialysis, conjugated estrogens
148
Factor V Leiden
mutation disrupts cleavage site on activated protein C, slowing the degradation of factor Va and factor VIIIa, increasing risk of venous thrombosis
149
Prothrombin G20210A mutation
mutation stabilizes prothrombin mRNA, increasing prothrombin levels, increasing risk of venous thrombosis
150
Antithrombin deficiency
mutation affects antithrombin protein that inhibits serine proteases such as thrombin and factor Xa, resulting in increased risk for thromboembolism
151
Protein C deficiency
mutation affects protein C, a serine protease that inactivates factors Va and VIIIa, resulting in increased risk for thromboembolism
152
Protein S deficiency
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
Acquired thrombophilic conditions
surgery, trauma, cancer, antiphospholipid syndrome
154
Antiphospholipid syndrome
acquired autoimmune disorder that induces tissue factor expression, disrupts protein C and antithrombin function, and activates platelets and the complement cascade
155
Function of heparin
binds to antithrombin and accelerates the inhibition of activated serine proteases factor Xa and thrombin
156
How is heparin cleared
UFH by endothelial cells and macrophages, while LMWH is renally cleared
157
Treatment for ductal carcinoma in situ breast cancer
lumpectomy or mastectomy with tamoxifen if estrogen receptor postive
158
Treatment for invasive breast cancer
lumpectomy or mastectomy, with sentinel node biopsy, followed by whole breast radiation
159
High risk for ovarian cancer
positive for BRCA or HNPCC should have prophylactic bilateral salpingo-oophorectomy before age 40
160
Signs of ovarian cancer
postmenopausal women with abdominal distention, pain, nausea, bloating, anorexia, ascites
161
Monitoring after ovarian cancer treatment
CA125
162
Testicular cancer tumor markers
elevated alpha fetoprotein defines a non-seminoma. beta HCG in both seminoma and non-seminoma, and serum LDH indicates rapid cell turnover.
163
Who gets non-Hodgkin lymphoma
men more than women, older age, more common in those who are immunosuppressed
164
Who gets Hodgkin lymphoma
age 15-45 and >55
165
Biopsy required for diagnosis of lymphoma
excisional biopsy rather than fine needle aspiration
166
Follicular lymphoma
median age 60, men and women equally, delay treatment until symptomatic. treat with rituximab +- CVP or CHOP
167
MALT lymphoma
associated with H pylori, remission with treatment of infection
168
Chronic lymphocytic leukemia
median age 70, delay treatment until symptomatic, treat with rituximab + fludarabine
169
Hairy cell leukemia
older adults, men more commonly, with progressive cytopenia and splenomegaly without lymphadenopathy. treat with cladribine
170
Diffuse large cell lymphoma
B and T cell types. B cell lymphoma with lymphadenopathy and fever, night sweats and weight loss
171
Superior vena cava syndrome
most commonly caused by lung cancer, causes dyspnea, facial swelling, cough, distension of neck veins, facial edema, cyanosis, facial plethora
172
Tumor lysis syndrome
lysis of tumor cells causes hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia, DIC and acute kidney failure
173
Malignancy associated hypercalcemia
nausea, vomiting, constipation, polyuria, polydipsia, weakness, confusion
