Hematology Flashcards
1
Q
What is acute lymphocytic leukemia (ALL)?
A
CHILD + lymphadenopathy + bone pain + bleeding + fever in a CHILD, bone marrow> 20% blasts in bone marrow
- population:children - most common childhood malignancy peak age 3-7
- highly responsive to chemotherapy (remission>90%)
2
Q
What is chronic lymphocytic leukemia (CLL)?
A
middle age patient, often asymptomatic (seen on blood tests), fatigue, lymphadenopathy, splenomegaly
- population: adults - most common from of leukemia in adults - peak age 50 y/o
- diagnostic studies: SMUDGE CELLS on peripheral smear, mature lymphocytes
- treatment with observation, if lymphocytes are >100,000 or symptomatic, treat with chemotherapy
3
Q
What is acute myeloid leukemia (AML)?
A
BLASTS + AUER RODS in ADULT PATIENT
- population: adults (80%) majority of patients >50 y/o
- anemia, thrombocytopenia, neutropenia, splenomegaly, gingival hyperplasia and Leukostasis (WBC >100,000)
- Aur Rods and >20% blasts seen in bone marrow
4
Q
What is chronic myeloid leukemia (CML)?
A
strikingly increases WBC count >100,000 + hyperuricemia + adult patient (usually >50 years old)
- population: adults - patient usually >50 y/o
- 70% asymptomatic until the patient has a plastic crisis (acute leukemia)
- diagnostic studies: Philadelphia chromosome (translocation of chromosome 9 and 22) “Philadelphia CreaM cheese”, splenomegaly
5
Q
What is anemia of chronic disease?
A
normal or decrease MCV, decrease TIBC, increase ferritin (high iron stores), decrease serum erythropoietin
- diagnostic studies: normochromic/normocytic anemia initially
- treatment: erythropoietin and treat the underlying disease
6
Q
What is aplastic anemia?
A
the only anemia where all three cell lines are decreased, decrease WBC, decreased RBC, decreased platelets - will have normal MCV and decreased retic
-diagnostic studies: pancytopenia
7
Q
What is folate deficiency?
A
decrease folate, increase MCV (macrocytic anemia) - looks like B12 but no neurologic symptoms
8
Q
What is the population and diagnsotic studies for folate deficiency?
A
population: Alcoholics Diagnostic studies: -megaloblastic anemia -serum folic acid: low -Macro-ovalocytes and hypersegmented PMNs (pathognomonic) -elevated homocysteine, normal MMA
9
Q
What is the tx for folate deficiency?
A
- PO folic acid 1-5 mg/d (first line)
- avoid ETOH and folic acid antagonists (Bactrim, phenytoin, sulfasalazine
- green leafy vegetables, yeast, legumes, fruits, animal proteins
- prophylactic folate acid - pregnant/lactating women, contemplating pregnancy, sickle cell patient
10
Q
What is G6PD deficiency?
A
after infection or medciation (oxidative stress) in a African American male (x-linked) + Heinz Bodies and Bite Cells on a smear (damaged hemoglobin - G6PD protects RBC membrane)
- hemolytic anemia
- African, middle eastern, S. Asian populations
- flare triggers: fava beans, antimalarials, sulfonamides
- Diagnostic studies: Heinz Bodies and Bite Cells on smear
11
Q
What is the tx for G6PD deficiency?
A
- avoid potentially harmful drugs, monitor infection
- acute - blood transfusion
12
Q
What is hemolytic anemia?
A
Caused by the premature breakdown of RBCs
- autoimmune hemolytic anemia (+Direct Coombs test) - increase retic, increased LDH, decreased haptoglobin, and increased bilirubin (indirect)
- hereditary spherocytosis (+) osmotic fragility test, increase Retic, increase LDH, decrease haptoglobin and increase bilirubin (indirect) and the presence of spherocytes
- G6PD deficiency after infection or medication (oxidative stress) in an African American male (x-linked) + Heinz Bodies and bite cells on a smear (damaged hemoglobin - G6PD protects RBC membrane
- sickle cell anemia (very increase retic count + pain in African American male, hemoglobin electrophoresis: Hemoglobin S, Blood Smear: sickled RBCs, Howell-Jolly bodies, target cells
- Thalassemia very decreased MCV (microcytis and hypochromic) with a normal TIBC and ferritin, elevated iron and family hisotyr of blood cell disorder
13
Q
What is iron deficiency?
A
decrease MCV (microcytic), decrease MCH (hypo chromic), increase TIBC, decreased ferritin (best test, low iron stores), target cells, pica and nail spooning
14
Q
What are the characteristics of iron deficiency?
A
- most common anemia in the US
- always consider GI bleed
- associated with pica and nail spooning
- Diagnostic studies:
- microcytic hypothermic anemia
- low ferritin (best test)/Fe, high TI
- target cells
15
Q
What is the tx for iron deficiency?
A
FeSO$ 325 mg TID
-packed red blood cells when Hgb <8
16
Q
What is sickle cell anemia?
A
African American, pain, family history of blood disorder, hemoglobin electrophoresis: hemoglobin S, Blood smear: Sickled RBCs, Howell-Jolly bodies, target cells
17
Q
What is hemolytic anemia?
A
- Population: African Americans, presents in the 1st year of life
- Hemolysis, jaundice, splenomegaly, priapism, poor healing, pain/swelling hands and feet, acute chest syndrome, pigmented gallstones
- diagnosis: hemoglobin electrophoresis: Hemoglobin S
- blood smear: sickled RBCs, Howell-Jolly bodies, target cells
18
Q
What is the tx of sickle cell anemia?
A
Hydroxyurea
-vaccine: meningococcal, pneumococcal, H. influenzae, influenza
19
Q
What is thalassemia?
A
family history of blood cell disorder, microcytic hypo chromic, elevated iron
20
Q
What is beta thalassemia major?
A
- most severe, mediterranean descent, failure to thrive
- hemoglobin electrophoresis:Hemoglobin A2 and F
- treatment: transfusion dependent, iron chelation (deferoxamine)
21
Q
What is beta thalassemia trait?
A
- mild anemia, often misdiagnosed as iron deficient
- hemoglobin electrophoresis: hemoglobin A2
22
Q
What is alpha thalassemia?
A
- Chinese and Southeast Asians
- hemoglobin elctrophoresis: Hemoglobin H (H disease), hemoglobin Bart’s (hydrops fetalis), Hemoglobin A (trait)
23
Q
What is Vitamin B12 deficiency?
A
increase MCV >100 (macrocytic anemia), hyperhsegmented neurophils and normal folate, decreased vibratory and position sense
24
Q
What are the characteristics of Vitamin B12 deficiency?
A
- etiology: pernicious anemia (antibody to intrinsic factor), gastrectomy, vegans
- glossitis: smooth beefy, sore tongue, neurologic symptoms (poor balance, low proprioception)
- Diagnostic studies:
- megaloblastic anemia (MCV>100), hyperhsegmented neutrophils
- elevated serum MMA, elevated homocysteine
- pernicious anemia: Schilling test (less than 10% radio labeled vitamin B12 in the urine, normal results when repeated with the administration of intrinsic factor)
25
What is the tx for Vitamin B12 deficiency?
Lifelong IM B12: 1-3 ug/d (animal products, fortified cereal) for pernicious anemia
- IV Cyanocobalamin 1 mg IM daily x 7 d, then weekly x 4 wk, then monthly for life
- PO B12 1-2 mg PO daily for vegans and bariatric surgery
- years to deplete stores
26
What is aplastic anemia?
the only anemia where all three cell lines are decreased, decreased WBC, decreased RBC, decreased platelets - will have normal MCV and decreased retic
- loss of blood cell precursors = hyperplasia of bone marrow, RBCs, WBCs, and platelets without reticulocytosis
- causes: chemicals, drugs, radiation (ACE-I, sulfonamides, phenytoin, chemo, radiation)
- s/sx: severe pallor, weakness, petechiae, ecchymosis, mucosal bleeding, severe infection
27
How is aplastic anemia dx?
pancytopenia = decreased WBC, RBC, platelets; most accurate = bone marrow biopsy
28
What is the tx for aplastic anemia?
stop causative agent, RBC transfusion, bone marrow transplant, immunosuppressive agents
29
What is Von Willebrand Disease (vWD)?
the Von Willebrand factor is found in plasma, platelets, and the walls of the blood vessels
- when the factor is missing or defective, platelets cannot adhere to the vessel wall at the site of an injury
- as a result, bleeding does not stop as quickly as it should
30
What are the characteristics of Von Willebrand Disease?
- most common genetic bleeding disorder, autosomal dominant
- decrease von Willebrand's factor (vWF) and decreased Factor VIII
- patient may present with excessive bleeding after a cut or increased menstrual bleeding
- you can differentiate this from hemophilia by lack of Hemarthrosis, small amounts of superficial bleeding, common to have bleeding with minor injury and petechiae
31
What is the treatment for Von Willebrand Disease?
treat with DDAVP (desmopressin) or in cases of excessive bleeding a transfusion of concentrated blood clotting factors containing von Willebrand factor
32
What is Hemophilia?
a hereditary bleeding disorder caused by a deficiency in one of two blood clotting factors: Factor VIII (A) or factor IX (B_
33
What are the two forms of hemophilia?
- hemophilia A, which accounts for about 80% of all cases, is a deficiency in clotting factors VIII ("aight")
- hemophilia B is a deficiency in clotting factor IX (Christmas disease)
Remember: Hemophila A ="aight" and b comes after A which is factor NINE
34
What are the characteristics of hemophilia?
- X-linked recessive so will affect males (most of the time)
- hemarthrosis, bruising, and bleeding
- Increased PTT, normal PT and platelets, with decrease Factor VIII or decrease factor IX on assay
35
What is the tx for hemophilia?
treatment involves the replacement of Factor VIII or IX
36
What is the presentation of easy bruising?
when a patient presents with easy bruising the major workup should be looking for platelet dysfunction or some type of coagulation deficiency or medication
37
What are the causes of easy bruising?
- medications (aspirin, coumadin, Xarelto, Eliquis, NSAIDs)
| - genetics (hemophilia A, B, C, Von Willebrand's, idiopathic thrombocytopenic purpura)
38
What is the initial workup of easy bruising?
- CBC with platelet count
- peripheral blood smear
- promthrombin time (PT) and partial thromboplastin time(PTT)
39
How is easy bruising dx?
- prolongation of PT or PTT may reflect clotting factor deficiency
- presence of an inhibitor of a component of the coagulation pathway (including the presence in the circulation of direct oral anticoagulants inhibiting thrombin or factor Xa)
40
What are the pearls of easy bruising?
- mild platelet dysfunction can be causes by aspirin, P2Y12 inhibitors, or NSAIDs and is very common
- easy bruising with no other clinical manifestations and normal laboratory test results is probably benign
41
What are the ddx for hypercoagulable state?
The differential diagnosis can be remembered with the mnemonic PVCS:
- platelets: too many (usually more than 1 million/uL) or overactive (TTP, Heparin-induced thrombocytopenis (HIT), HUS, and HELLP)
- vascular injury: from plaques, trauma, or burns
- clotting factors: anti-clotting factors protein C, protein S, or antithrombin III deficient or not working
- Stasis and surgery
42
What is Virchow triad?
blood stasis, hypercoagulable state, and vascular injury
- genetic causes include antithrombin III deficiency, factor V Leiden (activated protein C resistance), protein C deficiency, protein S deficiency, dysfibrinogenemia, and abnormal plasminogen
- acquired hypercoagulable states: malignancy, pregnancy, nephrotic syndrome, ingestion of meds (estrogen), immobilization, myeloproliferative DZ, US/Crohn's. Behcet's syndrome, polycythemia vera, intravascular devices, DIC, hyperlipidemia, antiphospholipid syndrome
- Heparin can cause HIT, causing decrease platelets followed by platelet activation causing clotting and infarction
- Lupus anticoagulants: IgM and IgG immunoglobulin is seen in 5-10% of pt with SLE but MC without lupus or in those taking phenothiazines
43
What is Factor V Leiden?
(MC): procoagulant clotting factor - amplifies the production to thrombin a clot formation
- mutated factor V resistant to breakdown by activated protein C = hypercoagulability
- increased DVT and PE especially in young patients
- Dx: activated protein C resistance assay; normal PT/PTT
- Tx: LMWH bridge to warfarin; long term antithrombotic therapy not recommended
44
What is Protein C Deficiency?
vitamin K dependent anticoagulant liver protein that stimulates fibrinolysis and clot lysis (inactivates factor V and VIII) - potentiated by protein S
- increased risk recurrent DVT/PE; may have family hx
- Dx: protein C/S functional assay: decreased protein C/S activity levels
- at risk for skin necrosis on warfarin
- Tx: heparin and oral anticoagulation for life
45
What is Protein S Deficiency?
vitamin K dependent that is cofactor for activated protein C, which inactivates procoagulant factors Va and VIIIa = reducing thrombin generation
- VTE = risk
- Dx: protein C or S functional assay: decreased protein C or S activity levels
- Tx: heparin/oral anticoagulation for life
46
What is Antithrombin III Deficiency?
recurrent venous thrombosis and PE, repetitive intrauterine fetal death (IUFD)
- Antithrombin III = natural anticoagulant; inhibits thrombin (IIa), Xa and other proteases; potentiated by Heparin
- associated with VTE; first episode 20 to 30 yo
- asymptomatic pt requires anticoagulation only before surgical procedures
- Pt with thrombotic events require high dose IV heparin then oral anticoagulation indefinitely
47
What is Antiphospholipid Antibody Syndrome?
autoimmune; often associated with SLE; characterized by thromboses and recurrent spontaneous abortions
- autoantibodies react against platelet membranes, activating endothelial cells and platelets = complement-mediated thrombosis
- Dx: lupus anticoagulant, anticardiolipin, DRVVT test, prolonged PTT
- Tx: high dose IV heparin with thrombotic events then oral anticoagulation indefinitely
48
What is Hodgkin's Lymphoma?
painless lymphadenopathy + bimodal age distribution (15-35) and (>60)
- fever, chills, and nigh sweats for >1 month
- painless enlarged posterior cervical and supraclavicular lymph nodes Virchow's node
- CXR - mediastinal adenopathy
- Excisional biopsy of the lymph node shows Reed-Sternberg cells
49
What is Non-Hodgkin Lymphoma?
look for an immunocompromised (HIV) patient with GI symptoms and painless peripheral lymphadenopathy
50
What is the age of patients with Hodgkin's Disease Lymphoma?
bimodal peaks in 20's then in 50's
51
What is the age of patients with Non Hodgkin's Lymphoma?
>50 years old, increased risk with immunosuppression (ex HIV)
52
What is the cell type with Hodgkin's Disease Lymphoma?
Reed-Sternberg cells are pathognomonic - B cell proliferation with bilobed or multilobed nucleus "owl eyes"
53
What is the cell type with Non Hodgkin's Lymphoma?
B cell: diffuse large B cell
| T cell, natural killer cells
54
What is the lymph node involvement with Hodgkin's Disease Lymphoma?
painless lymphadenopathy: upper body lymph nodes: neck, axilla, shoulder, chest (mediastinum)
-contiguous spread to local lymph nodes: usually localized single group of nodes
55
What is the lymph node involvement with Non Hodgkin's Lymphoma?
peripheral, multiple lymph nodes: axillary, abdominal, pelvic inguinal, femoral
-non contiguous, extranodal spread: GI and skin most comon
56
What are the B symptoms with Hodgkin's Disease Lymphoma?
B symptoms are common - fever, weight loss, night sweats
57
What are the B symptoms with Non Hodgkin's Lymphoma?
B symptoms not common
58
What is the EBV Association with Hodgkin's Disease Lymphoma?
associate with EBV (40% of patients)
59
What is the EBV Association with Non Hodgkin's Lymphoma?
rare
60
What is the management with Hodgkin's Disease Lymphoma?
excellent 5-year cure rate (60%)
61
What is the management with Non Hodgkin's Lymphoma?
variable cure rate
62
What is primary polycythemia?
due to factors intrinsic to red cell precursors
63
What is polycythemia vera?
a malignancy of the bone marrow that results in the overproduction of red blood cells (primarily), but also can affect platelets, and white blood cells
64
What are the classic symptoms of polycythemia vera?
pruritus after hot baths, as well as swelling, burning pain, and rubor of the hands and feet (erythromelaigia)
-patients may also have gout due to increased cell turnover leading to hyperuricemia
65
What are the 4 H's of polycythemia?
Hypervolemia (increase RBC), histaminemia (increase histamine due to release from mast cells), hyperviscosity (increased hematocrit = increased viscosity), and hyperuricema (increased uric acid)
- elevated RBC count. hemoglobin, hematocrit (usually > 50)
- elevated RBC count, hemoglobin, hematocrit (usually > 50)
- thrombocytosis, leukocytosis may be present
- serum erythropoietin levels are reduced
- elevated vitamin B12 level
- hyperuricemia is common
- increased histamine - proposed mechanism for intense pruritus associated with this disorder
- positive Jak2 tyrosine kinase mutation
- bone marrow biopsy confirms the diagnosis
66
What is the tx for polycythemia?
treatment consists of repeated phlebotomy to lower hematocrit to <42%
- older patients (>60 years old) and those with prior thrombosis should be treated with a myelosuppressive agent, most commonly hydroxyurea with or without aspirin
- anagrelide may be used to decrease the platelet count
67
What is secondary polycythemia?
is caused by either natural or artificial increases in the production of erythropoietin
-altitude related, hypoxic disease-associated (COPD, sleep apnea), bloodletting, genetic, neoplasms (i.e. pheochromocytoma, liver tumors)
68
What is idiopathic thrombocytopenic purpura (ITP)?
autoimmune reaction to platelets usually after a viral illness (ITP is insidious and chronic)
- diagnosis of exclusion
- associated with HIV, HCV, SLE, CLL
- CBC normal except low platelets (+ Direct Coombs Test)
69
What is the tx for ITP?
- children supportive care (IVIG for refractory cases)
| - adults treat with prednisone
70
What is thrombotic thrombocytopenic purpura vs ITP?
different from ITP (ITP is insidious and chronic) from TTP which is an acute febrile disease with multi-organ thrombosis (hence the name "thrombotic" thrombocytopenia)
71
What is thrombotic thrombocytopenia (TTP)?
decrease platelets + anemia + schistocytes (RBC fragments) on smear
72
What is the cause of TTP?
- after drugs: quinidine, cyclosporine, and pregnancy
| - inhibition of ADAMTS13
73
What is the presentation of TTP?
- adults
| - purpura and "FAT RN" - fever, anemia, thrombocytopenia, renal failure, neurological symptoms
74
What is the dx and tx for TTP?
dx: CBC normal expect low platelets, schistocytes (RBC fragments) on the smear, (-) coombs test
- tx: steroids, plasmapheresis
75
What is hemolytic uremic syndrome (HUS)?
decreased platelets + anemia + renal failure (associated with E. coli 0157:H7 and diarrheal illness in a child)
76
What is the presentation of HUS?
- post-infection: E. coli or Shigella
- children
- severe kidney problems
77
What is DIC?
abnormal activation of the coagulation sequence, leading to the formation of microthrombi throughout the microcirculation
-the causes the consumption of platelets, fibrin, and coagulation factors
78
What are the characteristics of DIC?
- fibrinolytic mechanisms are activated, leading to hemorrhage
- therefore, bleeding and thrombosis occur simultaneously
- infection, obstetric complications, trauma, malignancy, shock
- decreased platelets, increased bleeding time, increased PT, increased PTT, (+) D-dimer
79
What is the tx for DIC?
cryoprecipitate, FFP, platelet transfusion (if <30,000), heparin, treat cause
