Hematology Flashcards
1
Q
Erythropoietin
A
synthesized in peritubular capillaries
2
Q
Reticulocyte count
A
measure of effective erythropoiesis; correct for degree of anemia
3
Q
Extramedullary hematopoiesis
A
Hematopoiessi outside bone marrow (e.g. spleen)
4
Q
Newborn physiologic anemia
A
drop in Hb due to replacement of HbF RBCs with HbA
5
Q
Pregnancy
A
Hb and Hct decreased
greater increase in plasma volume than RBC mass
6
Q
Anemia
A
normal oxygen saturation and arterial PO2
7
Q
MCV
A
average VOLUME of RBCs; useful for anemia classification
8
Q
MCHC
A
average Hb concentration in RBCs
9
Q
When is MCHC decreased? When is MCHC increased?
A
MCHC
- decreased in microcytic anemias
- increased in spherocytosis
10
Q
Thalassemias
A
- decreased in MCV
- increased in RBC count
11
Q
RDW
A
RBC size variation
- increased in iron deficiency
- decreased in anemia of chronic disease, sideroblastic anemia
12
Q
Mature RBC
A
anaerobic glycolysis; no mitochondria or HLA antigens
13
Q
Total iron binding capacity is increased
A
- in iron deficiency
14
Q
Total binding capacity is DECREASED
A
in anemia of chronic disease OR sideroblastic anemia
15
Q
% Saturation
A
- decreased in iron deficiency or anemia of chronic disease
16
Q
% Saturation is increased
A
sideroblastic anemia
17
Q
Serum ferritin is decreased
A
- iron deficiency
18
Q
Serum ferritin is INCREASED
A
anemia of chronic disease, sideroblastic anemia, normal thalassemia
19
Q
Microcytic anemia
A
- seen in iron deficiency (most common cause)
- anemic chronic disease
- thalassemia
- sideroblastic anemia
20
Q
Most common cause of iron defieicny in a child
A
Meckel’s diverticulum
21
Q
Iron deficiency in women < 50
A
Menorrhagia (heavy bleeding during menses)
22
Q
Iron deficiency man < 50
A
Most common cause is peptic ulcer disease
23
Q
Iron deficiency in men/women > 50
A
Colon cancer
24
Q
Stages of iron deficiency
A
- decreased ferritin, Fe, and % saturation
- increased TIBC, normocytic then microcytic anemia
25
Anemia of chronic disease
Most common anemia in malignancy and alcoholics
26
Alpha Thalassemia trait
Autosomal recessive
two alpha globin gene deletions
- normal Hb electrophoresis
27
HbH disease
- three alpha-globin gene deletions;
- hemolytic anemia
- four Beta-globin genes
28
Hb Bart's disease
four alpha globin gene deetions
| four gamma globin genes
29
B- thalassemia minor
Autosomal Recessive
- DNA splicing defect
- increased HbA2 and F
- idecreased HbA
30
B-thalassemia major
nonsense mutation with stop codon
- hemolytic anemia
- very elevated HbF
- increased HbA2
31
Sideroblastic anemia
- defect in mitochondrial heme synthesis producing ringed sideroblasts
32
Causes of sideroblastic anemia
- Alcohol
- Pyroxidine deficiency (may be due to isoniazid tx of TB)
- Lead poisoning
33
Lead poisoning
- Inhibits ferrochetolase
- ALA Dehydrase (can't convert ALA to porphobillogen??)
- Ribonuclease
34
Sx of lead poisoning in children
- Growth retardation
- Pb in epiphyses
- Abdominal colic
- Encephalopathy
35
Sx of lead poisoning in adult
Peripheral neuropathy
| Proximal renal tubule damage (Fanconi's syndrome)
36
Lab of Pb poisoning
- Course basophillic stippling RBCs
- decreased MCV
- increase in Pb levels
- increase in ALA
37
Vitamin B12 sources
Animal products (requires intrinsic factor reabsorption in terminal ileum)
38
Vitamin B12
transfers methyl group to homocysteine
- involved in proprionate metabolism
- end product of succinyl CoA
39
R factor
binds with B12 in mouth, removed by pancreatic enymes in small intestine
40
Pernicious anemia
autoimmune destruction of parietal cells
sx: chronic gastritis in body/fundus, acholrydia
- increase in gastrin
41
Causes of folate deficiency
- Alcohol (most common cause)
- Poor diet
- Drugs
- Malabsorption
- Pregnancy
- Goat milk
42
Drugs and Folate deficiency
- Alcohol
- Oral contraceptives
- Phenytoin
- Methrotrexate
- Trimethroprim
- 5-fluorouracil
43
Intestinal conjugase in folate metabolism
Inhibited by phenytoin
44
Jejunal uptake of monoglutamate of folate
Inhibited by alcohol and oral contraceptives
45
Dihydrofolate reductase
inhibited by methotrexate, trimethoprim
46
Thimydilate synthase
inhibited by 5-fluorouracil
47
Most common cause of increased serum homocysteine
Folate deficiency
48
Lab in B12/folate deficiency
- Pancytopenia
- Hypersegmented neutrophils
- Increased in homocysteine
49
Lab finds unique to B12 deficiency
- increased gastrin (pernicious anemia)
| - increased methylmalonic acid
50
Likely diagnosis: B12 reabsorbed after administration of intrinsic factor.
Pernicious anemia
51
Likely diagnosis: B12 reabsorbed after administration of antiobiotics
Bacterial overgrowth
52
Likely diagnosis?
| B12 reabsorbed after administration of pancreatic enzyme
Chronic pancreatitis
53
Hb and Hct after acute blood loss
- Initially normal Hb and Hct
| .9% saline uncovers RBC deficit
54
Aplastic anemia
- Drugs (e.g. phenylbutazone)
- Infection (e.g. parvovirus)
- Benzene
55
Lab findings for aplastic anemia
- Pancytopenia
- Hypocellular bone marrow
-
56
Anemia in renal disease
Normocytic
| - decreased EPO
57
Extravascular hemolysis
- Macrophage phagocytosis of RBCs
| - Increase in unconjugated bilirubin and urine UBG
58
Intravascular hemolysis
- Decrease in serum haptoglobin
- Hemoglobinuria
- Hemosideriuria
59
Congenital spherocytosis
- Autosomal dominant
- defectin spectrin
- Extravascular hemolysis
- Splenomegaly
60
Blood findings in spherocytosis
Normocytic anemia
- Dense RBCs
- Increase in MCHC
- INcrease in osmotic fragility
61
Blood findings in spherocytosis
Normocytic anemia
dense RBCs
Increase in MCHC
Increase in osmotic fragility
62
PNH
missing decay acceerating factor
- complement destruction RBCs
- neutrophils
- platelets
63
Sx of PNH
- Pancytopenia
- Hemoglobiuria
- Positive sugar water test and acidified serum test
64
HbSS
- Autosomal Recessive
| - Missense mutation (valine for glutamic acid; 6th positive B-globin chain)
65
Causes of sickling
- Increase in deoxyhemoglobin (hypoxemia and acidosis)
| - HbS > 60%
66
HbF
inhibits sickling
| hydroxyurea increases HbF
67
Pathophysiology of HbSS
```
Vasoclusive crisises
Hemolytic anemia ( Extravascular)
```
68
HbSS in children
Dactylitis (6 - 9 months)
| Streptococcus pneumoniae sepsis (dysfunctional spleen)
69
HbSS osteomyelitis caused by what microorganism?
Salmonella paratyphi
70
HbSS complications
```
Aplastic crisis (parvovirus)
Acute chest syndrome
Autosplenectomy
Calciym bilirubinate gallstones
Priapism
Asceptic necrosis
```
71
HbAS
- microhematuria from sickling in renal medulla
| - renal papillary necrosis
72
Hb electrophoresis
HbAS - HbA (55 - 60%)
HbS (40 - 45%)
HbSS - HbS 90- 95%
HbF - 5-10%
73
Blood findings in HbSS
Sickle cells
Target cells
Howell-Jolly bodies (nuclear remnants)
74
G6PD deficiency
```
X-linked recessive
Oxidant damage (due to peroxide) to Hb (e.g. primaquine, dapsone, fava beans)
```
75
Blood findings G6PD deficiency
```
Heinz bodies (denatured Hb; special stain)
Bite cells
```
76
Pyruvate kinase deficiency
- Decreased ATP
- RBCs dehydrated
- increase in 2,3 BPG (right shift of oxygen-blood curve)
77
Warm Autoimmune-hemolytic anemia
```
IgG
Extravascular hemolysis (e.g. SLE, drugs)
```
78
Cold auto-immune hemolytic anemia
```
IgM
Intravascular hemolysis (e.g. CLL, mycoplasma)
```
79
Pencillin HS rxn
IgG antibody against penicilin attached to RBC (type II hypersenstivity)
80
Methyldopa
drug alters Rh antigens;
| IgG antibody against Rh antigens (type II antibody)
81
Quinidine
drug IgM immune complexes
intravascular hemolysis
type III hypersensitivity
82
Lab findings for autoimmune hemolytic anemia
Positive direct Coombs
| spherocytes
83
Microangiopathic hemolysis
Mechanical damage causing intravascular hemolysis
84
Causes of macro/micro hemolysis
Aortic stenosis (most common cause0
DIC
TTP
HUS
85
Peripheral blood findings micro/macro hemolysis
Schistocytes
| Iron deficiency from hemoglobinuria
86
Malaria
Intravascular hemolysis correlates with fever
| Falciparum - ring forms and gametocytes
87
Leukemoid reaction
exaggerated WBC response to infection
| Usually due to infection
88
Leukoerythroblastic reaction
Marrow infiltrative disease peripheralizes myeloblasts/nucleated RBCs
89
Leukemoid reaction
exaggerated WBC response to infection; usually due to infection
90
Causes of leukoerythroblastic reaction
Bone metastais MCC
| myelofibrosis
91
Eosinophilia
Type I hypersensitivity (e.g. penicillin reaction)
| Invasive helminthic infection
92
Helminths not producing eosinophili
Pinworms
| Adult worms in ascariasis
93
Atypical lymphocytes
```
Mononucleosis
CMV
Toxoplasmosis
Viral hepatitis
Phenytoin
```
94
Mononucleosis
due to EBV
| EBV attaches to CD21 receptors in B cells
95
Clinical findings for mononucleosis
Exudative tonsillitis
Generalized lymphadenopathy
Hepatosplenomegaly
96
Lab findings for mononucleosis
Atypical lymphocytosis
| IgM heterophile antibodies against horse RBCs (MONOSPOT)
97
Lymphopenia
T-cell deficiences (HIV)
| combined B/T deficiency (adenine deficiency)
98
Lymphocytosis
Viral infections; whooping cough
99
Chronic myeloproliferative disease
Neoplastic stem cell disorder
Splenomegaly
Marrow fibrosis
Risk of leukemia
100
Examples of myeloproliferative disease
Polycythemia vera
Myelofibrosis
Myeloid metaplasia
101
Relative polycythemia
Decrease in plasma volume, increase in RBC count, normal RBC mass
102
Absolute polycythemia
Increase RBC count and RBC mass
103
Appropriate polycythemia
Hypoxic stimulus for EPO to generate RBCs
104
Causes of appropriate absolute polycythemia
Lung disease
Cyanotic heart disease
High altitude
105
Appropriate absolute polycythemia
Normal plasma volume
Increase in RBC mass
Decrease SaO2
Increase EPO
106
Inappropriate absolute polycythemia
No hypoxic stimulus for EPO
107
Causes of inappropriate polycythemia
Ectopic secretion EPO
| Polycythemia vera
108
Polycythemia vera
Increase plasma volume and RBC mass
normal SaO2
Decreased EPO
109
Ectopic EPO (renal cell carcinoma)
Normal plasma volume
Increase in RBC mass
Normal SaO2
Increase in EPO
110
Myelofibrosis myeloid metaplasia
Marrow fibrosis
Extramedullar hematopoiesis
Splenomegaly
111
Lab findings in myelofibrosis
Tear drop RBCs
| Dry bone marrow aspirate (marrow fibrosis)
112
Essential thrombocythemia
MPD with increase in abnormal appearing platelets
113
Myelodysplastic syndrome
severe anemia in elderly; 30% develop leukemia
| ringed sideroblasts
114
Benzene
associated w/ aplastic anemia
| acute leukemia
115
Leukemia by age
ALL: newborn
AML: 15- 60
CML: 40 - 60
CLL: > 60
116
Acute vs chronic leukemia
Acute: blasts > 30% in bone marrow
Chronic: blasts < 10% in bone marrow
117
Acute Myeloproliferative Leukemia
Auer rods in myeloblasts
118
Acute Promyelocytic leukemia
t(15; 17)
defect in retinoic acid
Rx: retinoic acid (increased maturation)
DIC
119
Acute monocytic leukemia (AML)
Gum infiltration
120
Chronic Myelogenous Leukemia
t(9; 22) of ABL proto-oncogene
Philadephia chromosome 22
Decrease leukocyte alkaline phosphatase score
121
ALL
early pre-B (80%)
CALLA (CD10)
TdT positive
CNS testicle involvement
122
Indicators of good prognosis of ALL
t(12; 21)`
123
Chronic Lymphoblatic Leukemia (CLL)
pts > 60 years
B cell neoplasm
- decrease in gamma-globulins
- Generalized lymphadenopathy
124
Adult T-cell Leukemia
HTLV-1
CD4 T-cells
skin infiltration
lytic bone lesions with hypercalcemia
125
Hairy cell leukemia
positive TRAP stain ("trap the hairy spider")
splenomegaly
Rx with purine nucleosides
126
Nodal sites
```
germinal follicles
B cells
paracortex
T cells
sinuses
histiocytes
```
127
Testicular cancer metastasis
to para-aortic lymph nodes
128
Stomach cancer metastasis
to supraclavicular lymph nodes
129
Phenytoin side effect
- ginigival hyperplasia
- atypical lymphocytosis
- nystagmus
- ataxia
130
Cat scratch disease
Bartonella henselaw
| granulomatous microabscesses
131
Follicular B-cell lymphoma
t(14, 18)
| overexpression of BCL-2 (anti-apoptosis gene)
132
Burkitt lymphoma
```
t(8, 14)
overexpression of c-myc
EBV association
common childhood Non-Hodgkin's Lymphoma
"starry sky appearance
```
133
Extranodal lymphomas
risk factors:
H-pylori (stomach)
Sjorgen's syndrome
134
Mycosis fungiodes
CD4 T neoplasm
skin lesions with Pautrier's microabcesses
- when spread to blood becomes Sezary syndrome
135
Sezary syndrome
leukemic phase of Mycosis fungiodes
136
Polyclonal gammopathy
sign of chronic inflammation
137
Monoclonal gammopathy
```
M component (spike)
sign of plasma cell disorder
```
138
Bence Jones proteins
light chains in urine
| - predicive of a malignant plasma cell disorder (e.g multiple myeloma)
139
Multiple myeloma
patients > 45
- M spike
- lytic bone lesions
- pathological fractures
- hypercalcemia
- renal failure
140
MGUS
monoclonal gammopathy, may progress to myeloma
141
Findings in MGUS
elderly patient
no Bence Jones proteins
no malignant plasma cells
142
Waldenstrom's macroglobulinemia
lymphoplasmacytic lymphoma
IgM M spike (vs. IgG and IgA in multiple myeloma)
Hyperviscosity (b/c IgM are large molecules)
143
Hodgkin's lymphoma
neoplastic compnent Reed Stenberg cell ("owl eyes")
CD15 + and CD30+
- bimodal distribution: young adulthood or > 55 years
- EBV association
- Constitutional symptoms (e.g. weight loss, night sweats)
144
Reed Steinberg cell
- seen in Hodgkin's lymphoma
- binucleate or bilobed halves as mirror images
- more RS cells present the worse the prognosis
145
Non-sclerosing Hodgkins
- female dominant
| - supraclavicular nodes + anterior mediastinal nodes
146
Mixed cellularity Hodgkins
male dominant
numerous RS cells
EBV association
