RBC and Bleeding, Lung and Pleura, Head and Neck Flashcards
Differentials for microcytic anemia (MCV <80 fL)
TAILS
*Thalassemia
*Anemia of chronic inflammation
*Iron deficiency anemia
*Lead poisoning
*Sideroblastic anemia
Splenomegaly in the case of hemolytic anemia can be seen in what type of hemolysis?
*Extravascular hemolysis
*Chronic hemolysis
For intravascular and acute, usually hemoglobinemia, hemoglobinuria, hemosiderinuria due to hemoglobin being liberated inside the blood vessel (soon be cleared by kidney)
Defect in hereditary spherocytosis
Intrinsic - defect in membrane skeleton (spectrin, ankyrin, Band 3, Band 4.2) making them less deformable and easily lysed in spleen due to entrapment there
Etiology of G6PD
Intrinsic - G6PD deficiency –> ↓ NADPH –> ↓ reduced glutathione –> ↑ RBC susceptibility to oxidative stress
Triggers: infections, drugs, foods
Cell findings in G6PD
Heinz bodies (precipitated denatured Hb) - asplenic
Bite cells - intact spleen
Spherocytes
Etiology of Sickle cell disease
Autosomal recessive (SA is asymptomatic, SS is symptomatic)
Intrinsic: 6th codon of B-globin chain has point mutation (glutamate –> valine) –> hemoglobin S (HbS)
Situations favoring sickling of RBCs in Sickle cell disease
↓ intracellular pH
↓ O2 tension
Lab findings in Sickle Cell Anemia
Microcytic anemia
Target cell
Sickle cell
↓ HbA
↑ HbS
Thalassemia general etiology and pathogenesis
Etiology
↓ Globin chains –> ↓ Hb synthesis –> anemia
Pathogenesis
↓ in one globin chain –> excess of other globin chain –>precipitate inclusions –> membrane damage –> apoptosis within marrow –> anemia
Thalassemia morphology and lab:
CBC:
PBS:
Skull X-ray:
Others:
CBC: Microcytic hypochromic
PBS:Poikilocytosis, anisocytosis, Target cells
Skull xray: Crew cut appearance
Secondary hemochromatosis (liver, pancreas, heart)
Etiology:
Beta Thalassemia
Alpha Thalassemia
Beta thalassemia:
B-globin mutations (2 copies in Chromosome 11)
Alpha thalassemia:
A-globin gene deletions (4 copies in Chromosome 16)
Beta thalassemia that is transfusion dependent
Beta thalassemia major (2 mutated)
*Beta thalassemia intermedia (variable mutated), transfusion in special situations
*Beta thalassemia minor (1 mutated), asymptomatic or mild
Alpha thalassemia trait, how many alpha globin gene deletions
2
HbH, how many alpha globin gene deletions
3
(B4)
Hydrops fetalis, how many alpha globin gene deletions
4
(γ4)
When does anemia occur in those with Beta thalassemia? How about Alpha thalassemia
Beta thalassemia: 6-9 months after birth (shift from HbF –> HbA)
Alpha thalassemia: At birth (need of alpha globin chains even for HbF)
Why is Alpha thalassemia less severe than Beta thalassemia?
Because it only misses B-globin chains (and γ-globin chains) which are more soluble than a chains –> lesser inclusions –> ↓ membrane damage –> ↓ apoptosis in marrow –> less severe anemia
Defect in Paroxysmal Nocturnal Hemoglobinuria
PIGA mutations –> ↓ GPI anchor –> ↓ CD55, CD59, C8 binding protein –> ↑ susceptibility to complement mediated lysis
Antibody in Warm-Antibody Type Autoimmune Hemolytic Anemia
IgG active at 37C
*Associated with SLE, drugs, CLL
Antibody in Cold Agglutinin Disease
IgM active at <37C
*Associated with infections of Mycoplasma pneumoniae, CMV, EBV, HIV, and CLL
Antibody in Paroxysmal Cold Hemoglobinuria
IgG Anti P (Donath-Landsteiner antibody)
Coomb’s test that detects antibodies present in patient’s serum
Indirect Coombs’ Test
Used for compatibility testing, bloodbank etc.
*Direct Coombs’ Test detects antibodies attached to RBCs
ex: Hemolytic disease of fetus and newborn
Type of hemolysis in Cell finding in Hemolytic Anemia 2ndary to red cell trauma
Intravascular due to sheer stress
Cell finding in Hemolytic Anemia 2ndary to red cell trauma
Schistocytes (fragmented red cell)
Echinocytes (Burr cell)
General pathogenesis of megaloblastic anemia
Impaired DNA synthesis –> ineffective hematopoiesis –> premature death of precursor cells –> Peripheral blood cytopenias
*Morphology: Hypercellular marrow with megaloblastoid changes
Chem finding in Vitamin B12 deficiency (Pernicious anemia)
↑ Homocysteine
↑ Methylmalonate
Between Vitamin B12 deficiency and Vitamin B9 deficiency, __________________ has neurologic symptoms
Vitamin B12 deficiency
Chem finding in Vitamin B9 deficiency
↑ Homocysteine
NORMAL Methylmalonate
Iron studies: IDA
↓ Fe
↑ TIBC
↓ Ferritin
Iron studies: Anemia of Chronic Inflammation
↓ Fe
↓ TIBC
↑ Ferritin
Normal values
MCV
MCH
MCHC
MCV: 80-100
MCH: 26-32
MCHC: 32-36
Variability in red cell size
Anisocytosis
Variability in red cell shape
Poikilocytosis
Etiology and pathogenesis of Thrombotic Thrombocytopenic Purpura (ITP)
Deficient ADAMTS13 —> inc vWF polymers —> inc platelet activation —> inc thrombosis —> microangiopathic hemolytic anemia
Clinical features of TTP
FATRN
Fever
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal failure
Neurologic deficits
Etiology of ITP
Anti GpIIB/IIIA and Gp1b/IX IgG
HUS pathogenesis
Typical: E. Coli O157:H7 (Shiga-like toxin)
Atypical: complement activation inhibitors deficiency
*thrombocytopenia, anemia, renal failure
*Normal ADAMTS13 levels
DISORDER OF PLT FXN: Autosomal recessive inherited GpIIB/IIIa deficiency (problem in plt aggregation)
*inc BT, N PC
Glanzmann Thrombastenia
DISORDER OF PLT FXN: Autosomal recessive inherited GPIB/IX deficiency (problem in plt adhesion)
*inc BT, dec PC
Bernard-Soulier Syndrome (BSS)/Giant Platelet Syndrome
Most common inherited bleeding disorder
Von Willebrand Disease
(Autosomal dominant)
vWF factor deficiency —> FVIII deficiency
*inc BT, inc aPTT, normal PT
Most common hereditary disease associated with life-threatening complications
Hemophilia
(X-linked recessive)
A: Factor VIII deficiency
B: Xmas dse; Factor IX deficiency
C: Factor XI deficiency
Normal BT and PT, inc aPTT
Treatmed for vWD
Desmopressin
Factor VIII replacement
Heyde syndrome triad
Aortic stenosis
GI bleeding
Acquired vWD
DIC + Giant Hemangiomas
Kasabach-Merritt syndrome
Bilateral adrenal hemorrhage in meningococcemia
Waterhouse-Friderichsen syndrome
Acute DIC manifestation
Bleeding
Chronic DIC manifestation
Thrombosis
Most common indication for lung transplantation
Idiopathic Pulmonary Fibrosis
Masson bodies
Cryptogenic Organizing Pneumonia
Particle size most dangerous for pneumoconiosis
1-5 um (smaller particles can reach distal airways)
Exposure to what element causes silicosis
Silica (Quartz)
*building
*road demolition
*stone carvers
*jewellers
Exposure to roof insulation predisposes to
Asbestos
Silicosis affects the ________ lobes of the lungs while asbestosis affects the __________ lobes of the lungs
Upper; lower
Laminated concretions seen in sarcoidosis
Schaumann bodies
Stellate inclusions seen in sarcoidosis
Asteroid bodies
Most common organs involved in sarcoidosis
Lymph nodes (hilar and mediastinal) > lung
A lung condition that can look like pneumonia but does not resolve with antibiotics
Acute hypersensitivity pneumonitis
Macrophages with dusty brown pigment seen in smoking-related interstitial diseases (Desquamative interstitial pneumonia and respiratory bronchiolitis associated interstitial lung disease)
Smoker’s macrophages
WHO classification of pulmonary HTN secondary to autoimmune dse (BMPR2 mutations)
Group 1
WHO classification of pulmonary HTN secondary to unclear and multifactorial mechanisms
Group 5
Pulmonary hypertension has a mean pulmonary artery pressure of _________ mmHg at rest
> = 25 mmHg
Most common bacterial cause of acute exacerbations of COPD, otitis media and pink eye in children
Haemophilus influenzae
Bacterial cause of pneumonia in the elderly, and 2nd most common cause of acute exacerbations of COPD
Moraxella catarrhalis
Secondary bacterial pneumonia post-viral respiratory illnesses (measles and influenza)
Staphylococcus aureus
Most common cause of gram negative beacterial pneumonia, in chronic alcoholics, and with currant jelly like sputum
Klebsiella pneumoniae
Stage of lobar pneumonia
where the lungs are heavy
and boggy; Vascular
engorgement, intra-alveolar
fluid with few neutrophils,
numerous bacteria
Congestion
Stage of lobar pneumonia
where the lungs are red, firm,
airless with a liver-like
consistency. (+) intra alveolar
PMNs, RBCs, fibrin
Red hepatization
Stage of lobar pneumonia
where the lungs are grayish brown lobe, progressive RBC disintegration, with fibrinosuppurative exudate
Gray hepatization
Stage of lobar pneumonia
where exudate is broken down by enzymatic digestion into granular, semifluid debris
Resolution
Microscopic finding of the
lungs in bronchopneumonia
Neutrophilic rich exudate within airways
Most common symptoms of lung carcinoma
Cough > Wt loss > Chest pain > Dyspnea
Distant spread of lung carcinoma organs affected
ALBB
Adrenals
Liver
Brain
Bone
Most common mutations in nonsmokers with adenocarcinoma
EGFR
Adenocarcinoma mutation with worst prognosis
KRAS
IHC stain in lung adenocarcinoma
(+) TTF-1, Napsin A
Lung carcinomas that have a central site of predilection
Squamous cell carcinoma
Small cell carcinoma
Carcinoid/endocrine tumor
Lung carcinomas that have a peripheral site of predilection
Adenocarcinoma
Large cell carcinoma
Carcinoid/endocrine tumor
Mutations in Squamous cell carcinoma of lung
TP53, CDKN2A, FGFR-1
IHC stain in squamous cell carcinoma of lung
(+) p40, p63
Mutations in Small cell carcinoma of lung
(+) TP53, RB
Lung carcinomas with salt and pepper chromatin
Small cell carcinoma (inc mitosis)
Carcinoid/neuroendocrine tumor (dec mitosis)
Carcinoids arise from
Kulchitsky cells (neuroendocrine cells on basement membrane of bronchi/bronchioles)
A tumor that encases the lung
Diffuse pleural mesothelioma
> 95% of adult hemoglobin is
composed of this type
HbA1/HbA (Adult
hemoglobin): α2β2
Type of hemolytic anemia
that occurs due to inherent
defects in red cells
Intrinsic hemolysis
Type of hemolysis that occurs
in the spleen
Extravascular hemolysis
Clinical features observed in
intravascular hemolysis
Hemoglobinemia,
Hemoglobinuria,
Hemosiderinuria
Viral infection associated
with aplastic crisis
Parvovirus B19 infection
Most likely diagnosis in a
patient presenting with
jaundice and gallstones; Lab
findings include ↑ MCHC, ↑
RBC osmotic fragility
Hereditary Spherocytosis
Most common trigger of red
cell hemolysis in G6PD
deficiency
Infection
Precipitated denatured
hemoglobin seen in G6PD
deficiency
Heinz bodies
Having G6PD deficiency
and/or sickle cell trait is
protective against which
parasitic infection
Malaria
Pattern of inheritance of
sickle cell anemia
Autosomal recessive
Etiology of sickle cell anemia
Point mutation in the β-
globin gene (glu→val)
Most common cause of
morbidity and mortality in
sickle cell anemia
Vaso-occlusive (Pain)
crises
Autosplenectomy causes
increased susceptibility to
which type of organisms
encapsulated organisms (S.
pneumoniae and H.
influenzae)
Beta-thalassemia syndrome
with 2 mutated β globin
genes in chromosome 11
β- thalassemia major
Alpha-thalassemia syndrome
with 3 deleted α-globin gene
mutations
HbH disease
Alpha-thalassemia syndrome
with 4 deleted α-globin gene
mutations
Hydrops fetalis
Red cell surface proteins
absent in patients with
paroxysmal nocturnal
hemoglobinuria which
increases susceptibility to
complement-mediated lysis
CD55, CD59
Most common cause of
disease-related death in PNH
Venous thrombosis
Most common form of
immunohemolytic anemia;
Associated with autoimmune
diseases (SLE), Lymphoid
neoplasms (CLL)
Warm Autoimmune
Hemolytic Anemia
(WAIHA)
Type of antibody in WAIHA
IgG active at 37°C
Infections associated with
cold agglutinin disease
Mycoplasma pneumoniae,
EBV, CMV, HIV
Type of antibody in CAD
IgM active below 37°C
Disease-causing antibody in
paroxysmal cold
hemoglobinuria, which is
common in children postviral
infection
IgG anti-P (Donath-
Landsteiner antibody)
Possible deficient vitamins in
patients with megaloblastic
anemia
Vitamin B12, B9
Gastrectomy and
autoimmune atrophic
gastritis causes vitamin B12
deficiency due to the absence
of
Intrinsic factor
Parasite that competes with
vitamin B12 absorption
Diphyllobothrium latum
Features that differentiate
Vitamin B12 deficiency from
Vitamin B9 deficiency
(+) neurologic symptoms
due to subacute combined
degeneration of spinal cord
+ ↑ methylmalonate
(normal in Vit B9
deficiency)
DMARD used in the
management of RA that
causes Vitamin B9 deficiency
Methotrexate (a folate
antagonist)
Most common nutritional
disorder in the world
Iron deficiency anemia
Peptide hormone increased
in anemia of chronic disease
that prevents mobilization of
stored iron
Hepcidin
BMA: Hypocellular marrow
with fat cells and scattered
lymphocytes and plasma
cells; CBC:Pancytopenia with
normocytic, normochromic
anemia
Aplastic anemia
Most common cause of
aplastic anemia
Drugs
(+) Parvovirus B19
infection/autoimmune
disease; BMA: Absent
erythroblasts with normal
granulocytic and
megakaryocytic lineages
Pure red cell aplasia
Space-occupying lesions
displace bone marrow
elements
Myelophthisic anemia
Most common cause of secondary
myelophthisic anemia
Metastasis
A 5-year-old patient presents
with multiple petechiae. He
had a URTI 3 days ago and
has no significant medical
history. CBC: Low platelets
Immune thrombocytopenic
purpura (ITP)
Etiology of immune
thrombocytopenic purpura
Anti-GPIIb-IIIa and GPIb-IX
(80%) IgG
Form of ITP that is more
common in adults and is
associated with SLE, HIV, CLL
Chronic ITP
Thrombotic disease that
occurs due to decreased
ADAMTS13 level or activity
Thrombotic
thrombocytopenic purpura
(TTP)
Action of ADAMTS12
Degrades von Willebrand
factor (vWF) multimers
Causative agent of typical
hemolytic uremic syndrome
E. coli O157:H7
Autosomal recessive
inherited GPIb/IX deficiency;
defective platelet adhesion
Bernard-Soulier Syndrome
/ Giant Platelet Syndrome
Autosomal recessive
inherited GPIIb/IIIa
deficiency; defective platelet
aggregation
Glanzmann
Thrombasthenia
Most common inherited
bleeding disorder
Von Willebrand Disease
Clinical and laboratory
features of VWD
Mucocutaneous>Internal
bleeding, ↑ BT, ↑ aPTT,
Normal PT
Most common hereditary
disease associated with lifethreatening
bleeding
Hemophilia
Christmas Disease
Hemophilia B (Factor IX
deficiency)
Most common adverse
transfusion reaction
Febrile non-hemolytic
anemia
Most common reaction in
platelet transfusions
Allergic reactions
Transfusion-transmitted
infections are more common
in this blood product due to
its storage at room
temperature
Platelet concentrate
Type of atelectasis due to
airway obstruction
Resorption atelectasis
Type of atelectasis associated
with fluid, air, or mass inside
the pleural cavity
Compression atelectasis
Type of atelectasis that occurs
as a result of
pulmonary/pleural fibrosis
Contraction atelectasis
Abrupt onset of hypoxemia
and bilateral pulmonary
edema in the absence of heart
failure
Acute lung injury
Histologic hallmark for the
acute phase of acute
respiratory distress
syndrome
Diffuse Alveolar Damage
Irreversible enlargement of
airspaces distal to terminal
bronchiole
Emphysema
Type of emphysema
associated with smoking
Centriacinar
Type of emphysema
associated with α-1-
antitrypsin deficiency
Panacinar
Type of emphysema
associated with spontaneous
pneumothorax in young
adults
Distal acinar (Paraseptal)
Persistent cough with sputum
production for at least 3
months in at least 2
consecutive years in the
absence of any other cause
Chronic bronchitis
Ratio of thickness of the
mucus gland layer to the wall
thickness
Reid index (N = 0.4)
Chronic inflammatory airway
disease that produces
episodic variable expiratory
airflow obstruction
Asthma
Extruded mucus plugs seen in
sputum examination of
asthmatic patients
Curschmann’s spiral
Microscopic crystals made up
of galectin-10 from
eosinophils
Charcot-Leyden crystals
A known cystic fibrosis
patient presents with severe,
persistent cough with foulsmelling
mucopurulent
sputum. Episodes of
hemoptysis were also noted.
What is the most likely
diagnosis?
Bronchiectasis
Bronchiectasis, Sinusitis, Situs
inversus, Sperm dysmotility
Kartagener syndrome
Clinicopathologic syndrome
marked by progressive
interstitial pulmonary fibrosis
and respiratory failure; (+)
Honeycomb fibrosis
Idiopathic pulmonary
fibrosis (IPF)
Most important
environmental factor of IPF
Cigarette smoking
Black discoloration of the
bronchi due to carbon
deposits; accentuated in coal
workers’ pneumoconiosis
Anthracosis
Most common
pneumoconiosis and chronic
occupational disease
worldwide; (+) Eggshell
calcifications in lymph nodes
Silicosis
Hallmark of silicosis
Whorled collagen fibers
surrounded by dust-laden
macrophages
Most common manifestation
of asbestosis
Pleural plaques
Golden brown rods with
translucent core containing
asbestos coated with an ironprotein
complex
Asbestos bodies
X-ray finding in sarcoidosis
Bilateral hilar adenopathy
WHO classification of
pulmonary HTN secondary to
thromboembolism
Group 4
Most common cause of
community- acquired acute
pneumonia and lobar
pneumonia
Streptococcus pneumoniae
Bacteria associated with
hospital-acquired infections,
cystic fibrosis and
neutropenic patients
Pseudomonas aeruginosa
Viral pneumonia linked to
Parkinson’s disease later in
life
Influenza virus type A
Stage of lobar pneumonia
where the lungs are heavy
and boggy; Vascular
engorgement, intra-alveolar
fluid with few neutrophils,
numerous bacteria
Congestion
Stage of lobar pneumonia
where the lungs are red, firm,
airless with a liver-like
consistency. (+) intra alveolar
PMNs, RBCs, fibrin
Red Hepatization
Microscopic finding of the
lungs in viral pneumonia
Interstitial mononuclear
inflammation
Most common cause of lung
abscess
Aspiration
Most common primary lung
tumor
Lung carcinoma
Type of lung carcinoma
associated with
paraneoplastic syndromes;
strongest association with
smoking
Small cell carcinoma
Most common histologic type
of lung cancer; most common
type in never smokers;
peripheral in location
Adenocarcinoma
Type of lung cancer that is
central in location; (+)
keratinization, keratin pearls,
intercellular bridges
Squamous cell carcinoma
IHC stains positive in
carcinoid tumor
Synaptophysin,
Chromogranin
Cannon-ball lesions or
multiple discrete lesions in
the lung is typical of
Metastatic neoplasm
Most common cause of noninflammatory
transudative
effusions
Congestive heart failure
Mechanism of pleural effusion
in CHF
↑Capillary hydrostatic
pressure
Type of effusion associated
with radiation, autoimmune
disease, uremia
Serous, serofibrinous,
fibrinous
Type of effusion associated
with thoracic duct trauma
Chylous
Pneumothorax sufficient to
cause circulatory collapse due
to compression of mediastinal
structures
Tension pneumothorax
Type of pneumothorax that
occur due to rupture of
subpleural blebs in young
patients
Spontaneous idiopathic
pneumothorax
Most common pleural tumors
Secondary tumors (common
sources: lung, breast)
Most common cause of tooth
loss < 35 years old
Dental caries
Associated conditions with
recurrent/persistent
aphthous ulcers
Celiac disease,
Inflammatory bowel
disease, Behçet disease,
immunocompromised
Most common fungal
infection of oral cavity
Candida albicans
White pseudomembrane that
can be scraped off
Thrush
A white patch or plaque that
cannot be scraped off and
cannot be characterized
clinically or pathologically as
any other disease
Leukoplakia
Most common head and neck
cancer
Squamous cell carcinoma
Etiology of oral cavity SCCa
Tobacco, Alcohol, Betel
chewing, Sunlight, Pipe
smoking
Most common site of local
metastasis of oral cavity SCCa
Cervical lymph nodes
Etiology of oropharyngeal
SCCa
High-risk HPV
Odontogenic cysts located in
impacted/unerupted tooth
Dentigirous cyst
Condition associated with
multiple odontogenic
keratocyst
Gorlin syndrome (Nevoid
basal cell carcinoma
syndrome)
Odontogenic cyst associated
with history of dental
caries/trauma; location: apex
of non-viable tooth
Radicular cyst
Most common odontogenic
tumor
Odontoma
A patient presents with a
mass on the right mandible
in the posterior molar area.
Biopsy: epithelial cells
palisading around a stellate
reticulum.
Ameloblastoma
Most common cause of
bacterial
pharyngitis/tonsillitis
Group A beta-hemolytic
streptococci (GABHS)
An adolescent male presents
with recurrent epistaxis and
nasal obstruction. A mass
was seen in the posterior
roof of the nasal cavity
Sinonasal tract
angiofibroma
Virus associated with
nasopharyngeal carcinoma
(NPCA)
Epstein-Barr virus
Most common location of
NPCA
Rosenmuller fossa
Most radiosensitive type of
NPCA
Undifferentiated NPCA
Laryngeal lesion associated
with voice abuse
Reactive nodules
Most common benign
laryngeal epithelial tumor
Squamous papilloma
Etiology of laryngeal
papilloma
Low-risk HPV (6 and 11)
Common bacterial causes of
acute otitis media
S. pneumoniae, H.
influenzae, M. catarrhalis
Origin of branchial cleft cyst
2nd branchial arch
remnant
Most common cause of
xerostomia
Drugs (Anticholinergics,
Antihistamines,
Antipsychotics,
Antidepressants)
Most common inflammatory
salivary gland lesion
Mucocele
Salivary gland lesions that
occurs following sublingual
duct damage
Ranula
Most common viral
sialadenitis
Mumps
Bacterial sialadenitis can
occur in the presence of
Sialolithiasis
Most common salivary gland
tumor
Pleomorphic adenoma or
benign mixed tumor
Salivary gland tumor
associated with smoking;
Morphology: papillary
structures with central
lymphoid stroma covered by
double layer of oncocytic
cells
Warthin Tumor
Most common primary
malignant salivary gland
tumor
Mucoepidermoid
carcinoma
Most common location of
pleomorphic adenoma,
mucoepidermoid carcinoma,
and Warthin tumor
Parotid gland
A cancer arising from a
primary or recurrent
pleomorphic adenoma
Carcinoma ex pleomorphic
adenoma
Most common location of
adenoid cystic carcinoma
Minor (palatine) glands
Most common form of
adenoid cystic carcinoma
Cribriform
