Hematology-Panre Flashcards
1
Q
What is Microcytic anemia?
A
Iron deficiency anemia, Thalassemia, Lead poisoning and sideroblastosis (alcoholics)
2
Q
What is Thalassemia?
A
Think produces different types of shapes and sizes cells =poikocytosis
- Hereditary anemias in which alpha or beta globulin synthesis is reduced
- Results in defective hemoglobinization of RBCs (can’t get enough hemoglobin into cells)
3
Q
Types of thalassemia?
A
Alpha Major=homozyozous -death Alpha minor=severe anemia Beta major (recessive) =severe anemia( chronic anemia always needing blood transfusion lifelong) Beta minor =mild anemia
4
Q
How is Thalassemia dz?
A
Hb electrophoresis (Decrease HbA, Increase HbA2 & HbF)
Decrease Hgb, Severely decrease MCV (out of proportion low compare to hgb)
3. Normal TIBC, Ferritin, Retic count
4. Peripheral smear=HENIZ BODIES =ALPHA THALASSEMIA
5
Q
What is the treatment for Thalassemia?
A
- Blood transfusion
- iron chelation due to constant blood transfusion
- Splenectomy
6
Q
Most common anemia in the world
A
Iron Def
7
Q
What is Iron Deficiency Anemia
A
Caused by inadequate supply of iron (pregnancy and children) and blood loss (GI tract loss
and menstruating women
8
Q
MC cause of Iron def?
A
Blood loss GI tract (eg, peptic ulcer, cancer)
■ GYN (menorrhagia
9
Q
Causes of Iron def?
A
Blood loss (most common
Increased iron requirement (eg, pregnancy, infancy)
○ Decreased absorption (eg, gastrectomy, celiac disease
10
Q
Iron Deficiency Anemia s/s
A
Fatigue, SOB, pallor, weakness, dizziness, angina, pica, angular cheilitis,
glossitis, spoon/concave nails (koilonychia
11
Q
Iron Deficiency Anemia dx?
A
Microcytic, hypo chromic anemia, ↓ serum iron, ↓ serum ferritin, ↑ TIBC
12
Q
Iron Deficiency Anemia TX?
A
- Identify and treat underlying cause
2a. Oral iron: 60 mg elemental iron (eg, 325 mg ferrous sulfate) daily. Side effects include GI upset, constipation, dark stools.
2b. Ascorbic acid (500 mg) increases absorption - Transfusion if severe
13
Q
Will acute bleeding lead to microcytic anemia?
A
NO
14
Q
What are megalobastic anemia?
A
Big RBCS, little hgb Folate def Vitamin B12 (associate with pernicious anemia)
15
Q
What is Folate def?
A
A megaloblastic anemia due to deficiency in folate (Vitamin B9)
16
Q
RF of Folate Def?
A
- Inadequate intake: Alcoholism, malnutrition
- ↑ demand: Pregnancy, lactation
- Impaired metabolism: Folate antagonists (methotrexate, metformin),
anticonvulsants (phenytoin) 4. Impaired absorption: Celiac disease, gastric bypass
17
Q
s/s of Folate Def?
A
Fatigue, oral ulcers, glossitis, diarrhea, depression, confusion
○ Folate deficiency in pregnancy → neural tube defects
18
Q
How is Folate def dx with cbc?
A
macrocytic anemia, mild pancytopenia, HYPERSEGMENTED NEUTROPHILS;
↓ or borderline folate level; normal methylmalonic acid (MMA) level
19
Q
TX of Folate def?
A
Oral folate supplementation
○ Folate 400-1000 mcg/day
■ Normal daily requirement → 400 mcg
■ Pregnancy requirement → 600 mcg
20
Q
What is Vitamin B12 deficiency anemia (Level 2)
A
Deficiency in vitamin B12 due to inadequate absorption (eg,
inflammatory bowel disease, intrinsic factor deficiency) or inadequate intake (eg,
vegans) → results in megaloblastic anemia
21
Q
S/s of Vitamin B12 ?
A
Anorexia, diarrhea, glossitis, neurologic complaints (paresthesias, mental
sluggishness, shuffling gait), psychiatric complaints (depression
22
Q
Is Vitamin b12 reversible?
A
No
23
Q
How is Vitamin b12 dx?
A
Macrocytic anemia (MCV >100 fL), hypersegmented neutrophils, ↓ B12 level, ↑ SERUM METHYLMALONIC ACID (MMA) LEVEL, ↑ homocysteine levels
24
Q
Tx of vitamin b12
A
Vitamin B12 supplementation → 1000-2000 mcg PO daily
○ Severe vitamin B12 deficiency or neurologic symptoms → Vitamin B12 1
mg IM 1-4 times per week
○ Must differentiate from folate deficiency; folate supplementation can mask
B12 deficiency and worsen neurologic symptoms
25
Anemia + neuro =what type of anemia?
Vitamin B def
26
Normal or ↓ MCV, ↓ TIBC, ↑ Ferritin (high iron stores) ↓ serum erythropoietin
Anemia of Chronic dz?
27
Normochromic/normocytic anemia initially
Anemia of Chronic Disease
28
What is ITP
Autoantibodies are directed against platelet surface antigens, leading to premature platelet destruction;
29
What is the dx of exclusion of ITP?
Associated with HIV, HCV, SLE, CLL
| CBC normal except low platelets. (+ Direct Coombs Test)
30
Treatment of ITP ?
hildren supportive care (IVIG for refractory cases)
Adults treat with Prednisone (2/3 of pts respond, most within 1st week
o Impair clearance of Ab coated platelets
o Decrease auto-Ab production
o Improve integrity of leaky capillaries)
31
Where are petechiae found?
Areas of vascular congestion (e.g., below a tourniquet site or blood pressure cuff), dependent areas, areas exposed to constriction (tight clothing), and bony prominences
32
What are the 3 basic processes that result in thrombocytopenia?
Decreased production, increased destruction, and splenic sequestration
33
What is the pathophysiology of TTP?
Decreased ADAMTS13 enzyme activity, resulting in large uncleaved multimers of vWF causing platelet aggregation, microthrombi formation, and microangiopathic hemolytic anemia; ● vWF exposed
● Platelets adhere
● Enzyme used to cleave platelets from vWF is absent → local clot formation
● Leads to narrowed lumen which causes hemolytic anemia
34
:↓ Platelets + anemia + schistocytes (RBC fragments) on smear ?
TTP
35
What is the Pentad (TTP)
Thrombocytopenia, Anemia, Fever, ARF(acute renal failure), CNS; Mainly will have thrombyocyopenia , CNS, Anemia; ARF is rare
36
How is ITP different from TTP ?
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)
37
When should you suspect TTP?
Thrombocytopenia
o Microangiopathic hemolytic anemia (MAHA) – suspect with gross hematuria
o ± CNS
o Hematuria (may be only finding)
o No obvious cause but DO NOT WAIT FOR PENTAD
38
Who gets TTP?
```
Epidemiology
o Obese, African American, female, HIV
o Drugs: Quinine, clopidogrel, ticlopidine
o Untreated mortality >90%
o Treated mortality 10-20%
```
39
What are the labs of TTP?
CBC showing anemia, thrombocytopenia, fragmented RBCs (schistocytes), Increased unconjugated bilirubin ↑
LDH, ↓ haptoglobin, normal fibrin/fibrinogen negative direct antiglobulin (Coombs) test, ADAMTS13 level
<10% with presence of antibody against this enzyme
40
What is HUS?
MAHA, Thrombocytopenia, ARF(microangiopathic hemolytic anemia (MAHA)-no fever and cns (ttp for the kidneys); ↓ Platelets + anemia + renal failure (associated with E.coli O157: H7 and diarrheal illness in a child)
41
Presentation of HUS?
Post-infection: E.coli or Shigella
Children
Severe kidney problems ; Bacteria ingested → 3 days → non-bloody diarrhea → 2 days → pain & bloody!
● Platelet-fibrin clots form → low platelets, MAHA, ARF
● Send STOOL & URINE for Shiga toxin(symptoms are constant do not wax and wane like TTP-b/c they have fibrin)
42
HUS pathophysiology?
Shiga Toxin-Producing Escherichia coli (STEC): often due to UTI
o Endothelial damage → promotes thrombin generation
o Fibrin deposition → platelet-fibrin thrombi
43
Lab findings of HUS?
```
Schistocytes
● Thrombocytopenia
● Increased unconjugated bilirubin & LDH
● Normal fibrin/fibrinogen
● Negative Direct Coombs
● Negative blood culture for E. coli
```
44
Tx of HUS?
Supportive
● Admit for IVF
● Packed RBC for Hgb <6 or unstable vital signs
● Hemodialysis/peritoneal dialysis for anuria
● Resist giving platelets
● Antibiotics can increase toxin release
45
What is Heparin Induced Thrombocytopenia (HIT)
Defined as platelets <150K or >50% drop from baseline
● Type 1: hours to day after receiving heparin
● Type 2: 4-14 days after receiving heparin (mean 9 days)
● Delayed: 9-40 days (rare)
● Heparin works by blocking the conversion of Factor X to Factor Xa and conversion of
prothrombin to thrombin = prevents clotting cascade from forming stable fibrin clot
● LMWH blocks conversion of only Factor X to Factor Xa
● With HIT ® Ab formation takes away functional platelets
46
CM of HIT?
Venous thrombosis (30-70%) – DVT, PE, adrenal thrombosis, gangrene, cerebral or
cavernous sinus thrombosis
● Arterial thrombosis (15-30%) – limb ischemia, stroke, MI
Skin lesions (10%) – skin necrosis, erythematous plaques
● Acute reactions (10%)
● DIC (10%)
(4'T's Thrombocytopenia, Timing (5-14 days), Thrombosis, No oTher causes
47
HIT lab testing? TX?
```
Lab Testing
● Immunoglobulin assays
Treatment
● STOP HEPARIN
● No platelets
● No coumadin until platelet count normalizes
● Change to direct thrombin inhibitor
o Argatroban, Bivalirudin, Lepirudin (no longer available in US)
```
48
What is DIC?
PLATELETS CONSUMPTIONAbnormal activation of the coagulation sequence, leading to the formation of microthrombi throughout the microcirculation. This causes consumption of platelets, fibrin, and coagulation factors
49
DX OF DIC?
↓ Platelets, ↑ bleeding time, ↑ PT, ↑ PTT, (+) D-dimer
50
PATHOPHYSIOLOGY OF DIC?
Endothelial damage leading to:
o release of systemic cytokines like TNF, IL-6
o thrombin formation and fibrin deposition in usual manner
o anti-coagulation pathways become impaired
o clotting factors and platelets are consumed rapidly
51
IS DIC A PRIMARY DZ?
No, secondary to trauma, sepsis, malignancy, OB
emergencies, pancreatitis, liver failure, toxins, snake bites, drugs, transfusion reactions,
transplant rejections
52
Does thrombosis and bleeding occurs simultaneously?
Yes; Fibrinolytic mechanisms are activated, leading to hemorrhage. Therefore, bleeding and thrombosis occur simultaneously
53
Tx of DIC?
cryoprecipitate, FFP, platelet transfusion (if < 30,000), heparin, treat cause
54
Which thrombocytopenia causes increase of Pt/Ptt?
DIC, may get it with HIT
55
Which thrombocytopenia gets MAHA(hemolytic anemia)
ITP, HUS
56
Which thrombocytopenia has fibrin/fibrinogen abnormal?
DIC
57
Which thrombocytopenia causes Big spleen?
TTP
58
Which thrombocytopenia causes pt to look sick
TTP, DIC, possibly HUS
59
Which thrombocytopenia you can give platelets?
DIC, ITP
60
Which thrombocytopenia have clots formation (common)
HIT
61
Chronic Lymphocytic Leukemia (CLL) affects who? Also known as what? CM?
Population: Adults - most common form of leukemia in adults - peak age 50 y/o; Hairy cell leukemia -affects males>females ; often asymptomatic (seen on blood tests), fatigue, lymphadenopathy, splenomegaly
62
Chronic Lymphocytic Leukemia (CLL) is dx ?
SMUDGE CELLS on peripheral smear, mature lymphocytes
63
Chronic Lymphocytic Leukemia (CLL) Tx?
Treatment with observation, if lymphocytes are > 100,000 or symptomatic, treat with chemotherapy
64
What is leukemia?
ncludes a group of malignant disorders that affect the blood and blood-forming tissues of the bone marrow, lymph system, and spleen. The loss of cell division regulation leads to the accumulation of dysfunctional immature white blood cells (WBC) that, if left untreated, causes fatalities.
65
Acute lymphocytic leukemia (ALL) affects who? CM?
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-
66
Acute lymphocytic leukemia (ALL) tx?
Highly responsive to chemotherapy (remission > 90%)
67
What is Acute Myeloid Leukemia (AML): Cell type? Who does it affect? Origin cell type?
Cell type: More mature cells; Population adults; Origin : myeloid stem cells
68
Acute Myeloid Leukemia (AML) CM?
Anemia, thrombocytopenia, neutropenia. Splenomegaly, gingival hyperplasia and Leukostasis (WBC > 100,000)
69
Acute Myeloid Leukemia (AML) dx? TX? prognosis?
Aur Rods and > 20% blasts seen in bone marrow: chemotherapy; 60-70%
70
Chronic Myeloid Leukemia (CML): cell type? Population? Course?
More mature myeloid cells; Young-middle age adults; Transforms to acute leukemia (may be
stable many years and then transform
71
Chronic Myeloid Leukemia (CML): cell type? Population? Course?
More mature myeloid cells; Young-middle age adults; Adult patient (usually > 50 years old)Transforms to acute leukemia (may be
stable many years and then transform
72
Chronic Myeloid Leukemia (CML) Dx? Tx?
Increased WBC >100,000
| “Philadelphia Chromosome” (9; 22)
73
Chronic Myeloid Leukemia (CML) Dx? Tx?
Increased WBC >100,000
| “Philadelphia Chromosome” (9; 22); Chemo
74
What are lymphomas?
Group of disorders characterized by enlarged lymph tissue, spleen, and liver
75
Hodgkin Lymphoma cell type? Population? Nodes?
B cells; Age 15-45, after 50 (bimodal
distribution); Cervical, supraclavicular, ± mediastinal,
PAINLESS lymphadenopathy (except
pain post Et-OH ingestion)
76
Hodgkin Lymphoma Labs?
Reed Sternberg cells (owl eyes nucleus)
| seen on lymph node (LN) biopsy
77
Hodgkin Lymphoma prognosis?
Need CT C/A/P for staging, good
| prognosis (80% cure)
78
Hodgkin Lymphoma tx?
ABVD (adriamycin, bleomycin,
vinblastine, dacarbazine), localized
radiation vs. combined chemo
79
Hodgkins CM?
1 month. Painless enlarged posterior cervical and supraclavicular lymph nodes Virchow’s node. CXR - mediastinal adenopathy. Excisional biopsy of lymph node shows Reed-Sternberg cells
80
What is Non-Hodgkin Lymphoma? Cell ? population?
Group malignancies that arise from lymphocytes ; 90% for B lymphocytes; Greater incidence in HIV pts' <65 years old, increase incidence with
HIV
81
Non-Hodgkin Lymphoma Prognosis? TX?
Poor prognosis; Not as chemo sensitive, worse disease,
Tx based on stage
of disease (chemo, radiation, possible
bone marrow transplant after)
82
NOn-hodgkin CM? Dx?
Diffuse/painless lymphadenopathy
| generalized), Chest X-ray, need CT and biopsy,possible lumbar puncture, LDH for prognosis (tends to drop
83
What is Multiple Myeloma? MOA?
Malignant proliferation of plasma cells (plasma cells produce antibodies): Abnormal levels of monoclonal proteins are produced: IgG, IgA (can lead to renal failure
when clump in kidney)
84
Multiple Myeloma Dx-LABS; Xray?
Anemia,Rouleaux formation of RBC, Hypercalcemia
● Serum protein electrophoresis shows: MONOCLONAL spike (this is the protein secreted
by the tumor, Monoclonal proteinuria (Bence Jones Protein), Radiograph: X-ray showing lytic "punched-out" bone lesions of skull, spine, long bones
85
Multiple Myeloma CM?
Presentation: “CRAB”- Calcium elevation, renal failure, anemia and bone lesions. Anemia, bone pain, prone to infection;, Multiple myeloma patient that presents with LE weakness: suspect spinal cord
compression
● Hyperviscosity syndrome (can lead to organ failure, hypoxia);
86
MM tx?
Thalidomide (concern with flipper limb deformities in the fetus of pregnant mothers),
dexamethasone, doxorubicin
87
What is Myelodysplasia
are a group of diverse bone marrow disorders in which the bone marrow does not produce enough healthy blood cells
MDS is often referred to as a “bone marrow failure disorder”; Group of disorders characterized by peripheral cytopenia, dysplastic hematopoietic
progenitor cells, hyper- or hypo-cellular bone marrow → ↑ risk of acute myeloid leukemia
88
Myelodysplasia presentation?
yMay include: fatigue, weakness, shortness of breath, pallor, fever, infections,
↑ bleeding/bruising; more common with ↑ age
o Depends upon which cell line is most affected → anemia is most common; also,
neutropenia & thrombocytopenia
89
Myelodysplasia dx?
CBC, peripheral smear, bone marrow biopsy. Low RBCs, WBCs, platelets, normal/mildly elevated mean corpuscular volume (MCV), increased red cell distribution width (RDW); Low reticulocyte count, dysplastic RBCs, WBCs, normal platelets; ncreased blasts - 1–20% blasts
90
Myelodysplasia tx?
symptomatic patients my need transfusion; hematology referral; stem cell
transplantation is only curative tx
91
What is Hemochromatosis? Etiologies?
Abnormal accumulation of iron in parenchymal organs → multiple organ dysfunction;
most common cause of severe iron overload
● Etiologies:
o 1°: mostly autosomal recessive dz
o 2 °: hemoglobinopathies, excess oral intake, repeated blood transfusions
92
Hemochromatosis presentation? Dx?
may be asymptomatic until >40 yo; fatigue, skin hyperpigmentation,
diabetes mellitus, arthropathy, erectile dysfunction; other organ-related s/sx
● Dx: clinical; ferritin, transferrin saturation, genetic testing; serum iron & LFTs
93
Hemochromatosis tx? Related conditions?
Phlebotomy (1° tx for hereditary hemochromatosis), chelation tx
o GI / hepatology referral to r/o hepatic fibrosis or cirrhosis; Liver: cirrhosis, hepatocellular carcinoma
o Pancreas: “bronze” (hyperpigmentation) diabetes mellitus
o Cardiac: cardiomyopathy
94
polycythemia vera?
is 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
95
Polycythemia vera cm?
Classic symptoms include pruritus after hot baths, as well as swelling, burning pain, and rubor of the hands and feet (erythromelalgia); Patients may also have gout due to increased cell turnover leading to hyperuricemia
96
Polycythemia? Types
increase of hb and hct. Primary due to RBC progenitor cell mutation ex polycythemia vera. Secondary due to increase erythorpoeitin
97
POlycythemia vera dx?
clinical; CBC with diff, erythropoietin, genetic testing; Positive Jak2 tyrosine kinase mutation; Elevated RBC count, hemoglobin, hematocrit (usually >50), elevated uric acid
98
POlycythemia vera tx? complications?
phlebotomy (first-line; target Hct <45%), low-dose aspirin; hematology referral, bone
marrow biopsy; thrombosis (eg. TIA, stroke, DVT, Budd-Chiari syndrome), bleeding,
hyperuricemia; may develop iron deficiency (due to erythrocytosis), myelofibrosis or
acute myeloid leukemia
99
What is Thrombocytosis?
Platelet count >450,000/μL
o 1°: myeloproliferative neoplasms, myelodysplastic syndromes
o 2° (most common): reactive (eg. infections, inflammation, blood loss, postsplenectomy,
iron deficiency anemia
100
Thrombocytosis presents? dx? TX
Presentation: most pts are discovered incidentally with a finding of ↑ platelets
● Dx: clinical; CBC with diff, ferritin, CRP, genetic testing; other testing as appropriate to
exclude reactive processes
● Tx: directed at 2° causes; hematology referral, bone marrow biopsy
101
What is Transfusion reaction
Adverse event associated with transfusion of whole blood or one of its components →
ranges from minor to life-threatening
102
Acute Transfustion reaction -what occurs?
occurs during or soon after transfusion
o *Mild allergic/urticaria: hypersensitivity reaction
o *Febrile non-hemolytic: reaction to cytokines released from donor leukocytes
o Anaphylactic: severe allergic reaction
o Hemolytic: usually due to intravascular hemolysis from antigen-antibody
mismatch or incompatibility
o Septic: due to bacteria or bacterial byproducts in contaminated blood
o *most common
103
Delayed transfusion reaction?
can occur days to weeks later
o Delayed hemolytic: anamnestic response to prior antigen exposure (eg. prior
transfusion, pregnancy
104
Transfusion-associated circulatory overload (TACO
volume overload due to excessive
| volume of transfused components
105
Transfusion-related acute lung injury (TRALI
host immune response to donor human
| leukocyte antigen → pulmonary edema
106
Transfusion reaction CM ? Minor? Major?
urticaria, itching, fever (↑ >1°C), chills; major - dyspnea, ↑ or ↓ BP,
flank/back pain, AMS, jaundice, hemoglobinuria, oliguria, anuria, oozing from IV sites
107
Transfusion reaction tx?
Stop transfusion, maintain patent IV line; Reaction-specific treatment
§ Urticaria/febrile non-hemolytic: evidence for prophylactic
antihistamine/antipyretic is lacking
§ Hemolytic: aggressive hydration, diuresis
§ Anaphylactic: epinephrine, antihistamines, vasopressors
108
CML is common in what population?
Older males
109
Philadelphia chromosome (translocation of chromosome 9 and 22) Philadelphia CreaM cheese", splenomegaly
Chronic Myeloid Leukemia (CML)
110
Auer Rods and > 20% blasts seen in bone marrow
Acute Myeloid Leukemia (AML)
111
SMUDGE CELLS on peripheral smear, mature lymphocytes
CLL
112
ALL is common in what population?
children?
113
Most common acute form of leukemia? Common in what age population?
AML ; 70
114
Most common leukemia in adults
CLL
