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hemotologic disorder
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32
Pathology
Graduate
12/11/2016

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Term
Lymphoid hyperplasia (reactive)
Definition
no treatment > follow up, will resolve itself
large lmyphoid tissues in head and neck
1. waldeyer's rings
2. oral cavity lymphoid aggregates
3. other lymph nodes in neck

Histology: lymphocytes with germinal center, tnagile body macrophages
Term
reactive lymph node
Definition
common cervical nodes affected - atneriro cervical chain
> intrapartoid and buccal mucosa
two subtypes
A. acutely inflamed nodes: large, movable,tender, soft
b.Chronic node: enalrge, rubbery, non-tender
> diff dx: lymphoma
> HIV infection - >chronic lymph nodes
Term
Waldeyer's ring reactive hyperplasia lymphoid aggregates
Definition
tonsils asymmetric enlargement size varies
20 years old
large> touch midline > block airway
Term
Hyperplasia of oral lymphoid aggregates
Definition
most common: posterior lateral tongue (foliate papillitis)
> bilateral symmetrical, not ulcerated
> can look like carcinoma cuz location

other site: floor of mouth ,soft palate
[image]
[image]
Term
Hemophilia A (classic hemophilia)
Definition
factor 8 deficiency, X-linked recessive and abnormal PTT
males> affected, females are carriers
Variable expression: you can have this disease and 25% normal factor 8 still function normally. (<25% deadly)

often infected with HIV in the past: blood transfusion without filtration, gets AIDS
[image]
Term
Hemophilia B (Chirstmas disease)
Definition
factor 9 deficiency, x-linked recessive, abnormal PTT
Term
von willerbrand's disease
Definition
abnormal von-willebrand factors (lack), hence abnormal paltelets
most common of all blood inherited disease
no plasma glycoprotein> no adherence of platelets
no transport of factor * (von willebrand transport factor)
most common is mild, rare is signficiant
Term
Anemia
Definition
lossof blood by
1. bleeding
2. no hemoglobin
3. lack of Fe metabolism
megaloblastic anemia (lack of b12, foliate, macrocytic anemia)
hemolytic anemia
disorder hemoglobin
Term
Sickle Cell anemia
Definition
RBC sickel shaped hooking > false clot (thrombus) > gets stuck to blodo vessels = infarction
avoid lwo O2 tension: exercsise, high elevatio, pain
caused by: Beta globin chain having Glutamine switched with valine at 6th position.
less soluble when oxygenated than regular HbA
severity: depends on expressivitity
homozygous SC: severe (african american)
heterozygous: 50% hemoglobin affected is mild and common

Clinical : widened marrow psace/radicular seen on radiograph (hyperplastic marrow as compensatio for lack of good RBC)
[image]
Term
what's good about sickle cell blood?
Definition
you're resistant to malaria [image][image]
Term
Thalassemia
Definition
reduction of alpha globin/beta globin of hemoglobin
> reduced hemoglboin amount, ltos of globin chains in RBC
>Atypical RBC > spleen filtration (concentrate in spleen to be filerted) > splenomegaly causing
pt will have hypochromic microcytic anemia
also resistant to malaria
from incest, medieterarnea, african, indian, se asian
Term
Beta-thalassemia:
Definition
two genes expressing
a. 1 defective gene: minor >no clnical manifestoation
b. 2 defective tgenes: major thalassemia (Cooly's anemia)
- Fragile RBC... mcirocytic, hypochrmoci anemia, with hematopoiesis increased, widened marrow space (compensate, similar appearance to sickle cells)
maxilla and mandible enlargement and hair on end skull radiographically
BIRTH DEEATH[image]
Term
Alpha thalassemia:
Definition
4 genes all for alpha globin > more spectrum of disease
1 gene no dz
2 genes alpha thalassemia trait (carrier)
3 genes HbH disease (hemolytic anemia/splemomegaly)
4 genes affected hydrops fetalis (fe hours after birth death)
tx: do not mutliple transfuse... will die
Term
Neutropenia
Definition
lack of PMNs... below 1500/Mm3 (250-700 range)
cause: malignant bone marrow via virals or drug iducing
> high infection
> suppuration and abscess however is absent
> oral lesions/ulcers> attached gingiva> white, and necrotic
> ulcerated at tip of interdental papilla and tip of tongue [image][image]
Term
Cyclic neutropenia
Definition
rare, idopathic, healing comprosied in window
WIndow of disease is 3-6 days, have to diagnose at the right time to get cyclic neutropenia
PMN level decreases for 3-6 days then icnerase back to normal amount.

young peopel disease (childhood) > anorexia, fever, cervical lymphadenopathy, malaise, pahryngitis, oral ulcers... perio bone loss

tips of interdnetal papilla is necrotic/white, and ventral tongue pink circle
[image][image]
Term
Agranulotcytosis
Definition
granulocytic cells are absent
mostly drug caused... bacterial second
oral: necrotize, punched out ulcerations/gingivitis, full thickness of gingiva gone
[image]
Term
what is the difference between neutropenia and agraulocytosis?
Definition
neutropenia: see necrosis at tip of interdental papilla

agranulocytosis: loss of granulocytes, see punched out ulcerations of gingiva (full thickness)
Term
Thrombocytopenia
Definition
lack of platelets from
a. loss of production
b. destrcution
c. spleen filtration
d. die prematurely cells (normal 180 days)
> udner 100K/mm3 count to be symptomatic
petechiae, ecchymosis, hematoma formation
> cough, oral sex, osft palate dmage
> maorrow decreased

can involve with monomucleosis (kissing disease)
> soft/hard palate asthmatous
marrow consuming clot factor> gingival bleed
Term
Polycythemia Vera
Definition
rare, idiopathic hematologic
lots of RBC 2-3x rate
60 years old
blood high viscous, thrombus likely
itchy red skins, marrow active...
can develop to acute leukemia an clot formation
tx: donate blood [image][image]
Term
Leukemia
Definition
malignancy of bone marrow stem cells that gets into circulation
can be acute, chronic, or myeloid, or lymphocytic
> acute is very aggressive, chornic is indolent
histo: lots of leukocytes
[image]
CLinical: gingival red,ooze fromc rest/junction
> petechial hemorrhages, oral ulcer, fungal ifnection, herpes infection ,gingival enlargement.
Prognosis: dpeends on age, tyep and treament
there are 4 types [image]
Term
the four types of leukemia
Definition
Acute lymphocytic leukemia: most common form of childhood leukemia
acute myelogenous leukemia: most common tyype with oral involvement, 2nd most common in childhood, and most common form of adult leukemia
chronic lymphocytic leukemia - one of the most common form of adult leukemia
chornic lmyelogneous leukemia

most common childhood: ALL
second most common childhood: AML
most common oral manifestation :AML
most common adults :AML and CLL[image] [image][image]
Term
Hodgkin's disease
Definition
malignancy of lymph nodes > see a lot of reed-sternberg cells > similar to giant macrophage cells

swollen neck from cervical supraclavicular nodes
mostly male, 15-35 years, and after 50, afrrican american (bipedal age affected)
4 subtypes ( cellularity/histology dependent)

prognosis: depends on stage
nodular sclerosis - most common form
mixed cellularity
lymphocyte dominant
lymocyte depleted - least common [image]

lots of giant cells [image] [image]
Term
what are the 4 stages of hodgkin's disease?
Definition
stage 1: single lymph node
stage 2: 2 or more lymph nodes in the same side of diaphragm, locally extralymphatic
stage 3: lymph nodes on both sides of diagphram, spleen affected
stage 4: diffusive extralymphatic disease (affecting liver, bone marrow ,lung ,skin)
worst is stage 4
[image]
Term
Non-hodgkin's lymphoma
Definition
lymphoreticular malignancies of T and B cells
swollen lymph nodes, MUCH more aggressive than hodgkin's
MOST common form of lymphoma is non-hodgkin's.
head and neck region affected
two types:
B cell lymphoma: most commonly associated non-hodgkin's lymphoma
T cell lymphoma

adult only, espeically immnologic disorder> HIV/AIDS patients
also see bialteral cergivcal lymph node swells[image]
Term
oral lymphoma
Definition
soft tissue and bone affected, extranodal disease, non tender, swelling diffusion.
Head and neck: buccal vestibule, gingiva, and posterior hard palate
bone: vague pain, discomfort, jagged RL... similar to periapical RL
histo :lots of infiltration uniform neoplastic lymphocytes
radiograph similar to periapical RL[image]
[image] [image] [image][image][image][image][image] [image]
Term
oral lymphoma when under radiograph looks like... (aka differential dx based on radiograph)
Definition
periapical radiolucency

difference? teeth of oral lymphoma is vital
Term
Mycosis fungoides (cutaneous T cell lymphoma)
Definition
malignancy of T lymocytes seen in skin via epidermotropism
middle age 55-60
slow progressive and aggressive
3 stages:
eczematous: very similar to psoriasis
plaque: microscopic features
tumor: diffuse infiltration dermis, atypical T lymphocytes[image]
[image]
Term
Burkitt's lymphoma
Definition
aggressive B cell lymphoma
african children in maxilla (unilateral)
associated with epstein-barr virus
histo: starry sky, dense infiltration of B cells
osteorx: perforate lymphoma lesions
radiograph: eaten jaw, swelling, teeth displaced
Burkitt's americans' form tend to be seen in abdominal
African seen in jaw. [image][image][image] [image]
Term
Extranodal NK/T Cell lymphoma, nasal type midline lethal granuloma
Definition
aggresive destruction of midline... adult patients
T/NK cell lymphoma
6 months to live, prognosis poor
hard palate, max sinus affected/communicate with one another
painful... necrosis
histo: inflammatory cell loop aroudn blood vessels (angiocentric)
[image]
Term
there are 4 differential dx of hard palate/infiltration lesions that cross midline
Definition
1. deep fungal infection mucomicosis
2. Nk/T Cell lymphoma
3. cocaine user that's using it aggressively
4. Wegener's granulomatosis
Term
Multiple myeloma
Definition
plasma cell malignancy
most common primary tumor of bone
old patient, 70 years, black and male
jaws= fracture
radiograpH multiple punched out RL of jaw and skulls
histo: bence-jones proteins! > amyloides possible> affect kidneys
B cell lots of them
Bone marrow leaky> damaged wbc> multipel myeloma cells via M proteins
tx: alkaline, chemotherapy
Term
the process of multiple myeloma working is
Definition
monoclonal antiobdy/immunoglobulin production...
bsically one alpha or kappa globin chain on the immunoglobulin only. normal Ab has both chains.
thicker blood...

see low blodo count, hyeprcalcemia via urination, restless, confusion, thirst, nausea, imparied kidney: becne jones protein amyloidosis and ca in blood
[image]
[image]
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