Hematological diseases Dr. T Flashcards
What causes Iron Deficiency anemia?
- *menstruation, pregnancy, malabsorption diseases
(eg. Celiac)**
What causes Megaloblastic Anemias
?
o Folic Acid def
o Vit B12 def (pernicious anemia)
What causes Thalassemia & Sickle Cell anemia ?
Genetic
What causes Aplastic Anemia ?
– environmental, things like bensin
What causes Anemias of Chronic Renal Insufficiency?
low levels of
erythropoietin – organ disease
What causes Anemias of Chronic Disease?
inflammatory conditions, malignancy
– organ disease
What causes Myelodysplastic Syndrome?
– environmental
What is this clinical presentation?
microcytic,
hypochromic anemias
(iron deficiency):
Pallor
▪ Anemia: reduction in O2 carrying capacity
▪ Diagnosis: RBC counts, Hg, HCT, red cell indices for dx of type of
anemia
▪ Normal upper labial mucosa
▪ Pale lower labial mucosa
What is this clinical presentation?
Iron deficiency anemias
Microcytic hypochromic
▪ Atrophic glossitis – tongue has been a little denuded of papilla
▪ Angular cheilitis – white and redness at angle of mouth/
commissure
▪ Plummer‐Vinson Syndrome: type of iron deficiency anemia
o Chronic iron def, dysphagia (esophageal webs), atrophic glossitis.
- Significantly increased risk of esophageal cancer (SCC)
- More commonly seen in European women
- Have difficulty swallowing
What is this clinical presentation?
microcytic,
hypochromic anemias
(iron deficiency):
Pallor
▪ Anemia: reduction in O2 carrying capacity
▪ Diagnosis: RBC counts, Hg, HCT, red cell indices for dx of type of
anemia
▪ Normal upper labial mucosa
▪ Pale lower labial mucosa
What is this clinical presentation?
Macrocytic
(megaloblastic)
Anemias:
▪ Folic acid and B12 deficiencies
- Glossitis
- Denuded dorsal surface
- Burning, stinging pain
Seen in older patients and seen in patients with poor nutrition
Etiology: alcoholism, malabsorption, medications
(trimethoprim, oral contraceptives, anticonvulsants
What is Pernicious anemia?
What causes it?
Which age and race does it most affect?
Pernicious – “highly injurious or deadly”
▪ Lack of intrinsic factor protein in stomach decreases absorption of
vitamin B12
Etiology is usually the body making antibodies against parietal cells which make intrinsic factor
o Autoimmune disease effecting parietal cells leads to
decreased intrinsic factor
▪ More common in elderly
▪ More common in patients of Northern European and African
decent
This anemia can lead to death
If a patient has a Burning Tongue
What steps must we take
fungal (candidiasis) –> mucosal smear/ Empirically can give antifungals (to figure out if it is candidiasis can do a smear or give antifungal)
If not candidiasis order cbc
▪ Order CBC with diff
▪ Order blood levels on Fe, Folate, B12, zinc
▪ Rule out : diabetic neuropathy
If not candidiasis and blood work didn’t show anything abnormal patient has burning mouth syndrome
▪ TIBC tests to see if theres too much or too little FE in the blood,
measures the bloods capacity to bind fe with transferrin
▪ B12 def can be from pernicious anemia ( Schilling test for
Pernicious Anemia) lack intrinsic factor to allow proper absorption, malnutrition or malabsorption from GI conditions can lead to this as well.
What is the treatment for
Deficiency Anemias
Iron def?
▪ Ferrous sulfate 325 mg TID between meals
o can cause Constipation
▪ add on fiber to diet, green leafy vegetables
o IV doses for absorptive problems
▪ 125mg in 100ml saline infused over 1 hour
What is the treatment for
Deficiency Anemias
Folic Acid def?
o 1mg PO, 5mg in malabsorptive disease
o Pregnant women given folic acid to decrease spina bifida
o Can be given IM injections if have malabsorption
What is the treatment for
Deficiency Anemias
B12 def?
- b 12 1mg IM injections weekly for 1‐2 months
- Monitored for lifetime
What is Thalassemia?
▪ A group of disorders of hemoglobin synthesis characterized by
decreased synthesis of either the alpha‐globin or beta‐globin
chains of the hemoglobin molecule
Patients get a microcytic, hypochromic anemia
▪ People with the abnormal gene have a resistance to malaria
▪ People who have the trait rather than the disease are more likely
to live and not die from malaria and spread that gene to their
children
▪ Severity depends on the specific genetic alteration and whether it
is homozygous (severe) or heterozygous (no clinical sign to mild
manifestations)
What is this clinical presentation?
Beta‐thalassemia
▪ Two defective genes – thalassemia major (Cooley’s anemia,
Mediterranean fever)
o Disease detected when fetal hemoglobin ceases to be
made (~3‐4 months old)
o Extramedullary hematopoiesis cause hepatosplenomegaly,
bone marrow hyperplasia, and lymphadenopathy
o In jaws, painless enlargement of maxilla and mandible
(“chipmunk” facies)
o Skull films show “hair‐on‐end” appearance
o Untreated, patient dies by about one year of age
o Treatment is repeated blood transfusions or bone marrow
transplant
this pt was treated with
What is this clinical presentation?
Beta‐thalassemia
▪ Two defective genes – thalassemia major (Cooley’s anemia,
Mediterranean fever)
Hyperplasia of maxilla, body is trying to make more rbc so bone
marrow enlarges to support space of those red blood cells but rbc
are abnormal so spleen keeps destroying them
What type of Alpha‐thalassemia is not compatible with life
When there are Four defective genes – Hb Bart’s hydrops fetalis
o Lethal in utero or within a few hours of birth
o Not compatible with life
▪ Estimated that 5% of world population carries a variant of Alpha‐thalassemia (over 100 genetic forms)
▪ One defective gene –
o no disease detected
▪ Two defective genes – alpha‐thalassemia trait
o Mild degree of anemia (usually not clinically significant)
▪ Three defective genes – Hb (hemoglobin) H disease
o Hemolytic anemia and splenomegaly
Which ares alpha thalassemia is common?
Areas with a lot of malaria
What is Polycythemia
▪ Increase in the number of circulating red blood cells
What are the types Polycythemia ?
▪ Types
- Primary polycythemia: polycythemia vera
- Secondary polycythemia: response to low O2 environment such as sleep apnea, living in high elevations, smoking
-
Relative polycythemia
- Dehydration, diuretics, vomiting
People with chronic Polycythemia are at
increased risk of —— or ——-
- *of MI or CVA (cerebral vascular accident or
stroke) **
What is this clinical presentation?
Secondary
Polycythemia
Oral manifestation
▪ Oral mucosa appears deep red
▪ Glossitis
▪ Gingiva appears edematous and bleeds easily
▪ Consequent “crowding out” of WBCs and platelets may result in
other manifestations
What is Sickle Cell Anemia?
Type of inheritance
What causes it?
▪ Genetic disorder of hemoglobin synthesis (one of the
hemoglobinopathies)
▪ Autosomal recessive pattern
▪ Mutation in DNA of thymine for adenine causes alteration of
codon resulting in the substitution of the amino acid valine for
glutamic acid in the beta‐globin chain of hemoglobin
▪ This transformed hemoglobin is prone to molecular aggregation
and polymerization to form a rigid and curved shape (sickle shape)
What is Sickle cell crisis
What trigger it?
What are the symptoms?
effect on dental tx?
consequences?
- sickling of the RBCs becomes severe
- precipitated by such things as hypoxia, infection,
hypothermia, or dehydration - Symptoms include pain (which may be severe) due to ischemia
and infarction of affected tissues
▪ Long bones, lungs and abdomen are common sites of occurrence for “crises”
▪ Episodes last from 3‐10 days, some patients have monthly crises, some may go a year or more between
▪ May need pre‐medication prior to dental treatment -
infections are the most common cause of death in sickle cell
patients (in the US)
▪ Patients also have impaired kidney function and CNS involvement (strokes in 5‐8%, often prior to age of 10)
What is Sickle cell crisis
treatment and how it diagnosed?
- ▪ Transcranial Doppler scan used to evaluate blood flow in brain
▪ Hydroxyurea may be used as a therapy
o Induces Hb F formation (doesn’t sickle)
o But can be carcinogenic and teratogenic
Sickle Cell Anemia Types and which is resistant to malaria?
- Sickle cell trait ‐ one allele is affected, only 40‐50% of hemoglobin will be abnormal, no significant clinical manifestations
- Sickle cell disease ‐ both alleles affected, close to 100% of hemoglobin is abnormal, significant clinical problems
▪ Abnormal gene confers resistance to malaria, therefore, sickle cell trait is seen most frequently in populations from areas endemic for malaria (Africa, the Mediterranean, and Asia)
What is this radiographic presentation?
Sickle Cell Anemia
Radiographic findings include:
o decreased trabecular pattern in the mandible (due to
increased extramedullary hematopoiesis)
o laddering of inter‐radicular trabeculae
o “hair‐on‐end” appearance of skull films (less prominent
than with thalassemia)
Can see spontaneous pulpal necrosis in the absence of trauma or
caries
What is this clinical presentation?
Sickle Cell Anemia
Can see prominent trabeculae left, looks like steps on a ladder
What is Aplastic Anemia?
What it is associated with?
Who are at increased risk of developing this anemia?
▪ Failure of the hematopoietic precursor cells to produce adequate numbers of all types of blood components
▪ Rare, but life threatening
▪ Associated with environmental toxins (benzene), certain drugs
(antibiotic chloramphenicol), or infection with certain viruses
(non‐A, non‐B, non‐C, non‐G hepatitis)
▪ Fanconi’s anemia and dyskeratosis congenita patients have an
increased risk of developing this
What is this clinical presentation?
Aplastic Anemia
▪ Failure of the hematopoietic precursor cells to produce adequate
numbers of all types of blood components
▪ Rare, but life threatening
▪ Associated with environmental toxins (benzene), certain drugs
(antibiotic chloramphenicol), or infection with certain viruses
(non‐A, non‐B, non‐C, non‐G hepatitis)
▪ Fanconi’s anemia and dyskeratosis congenita patients have an
increased risk of developing this
Oral signs: are associated with the thrombocytopenia and include
spontaneous gingival hemorrhage, mucosal petechiae, purpura,
and ecchymoses
▪ Nonspecific oral ulcerations (neutropenic ulceration) may also be seen on any mucosal surface, but especially in areas of trauma
(even minor trauma, ex. gingiva)
o Neutropenic = low neutrophils
Aplastic Anemia
treatment
supportive care initially, attempts to stimulate bone marrow (androgenic steroids), and bone marrow transplants for severe cases (prognosis is guarded at best)
What is this clinical presentation?
Aplastic Anemia
- One of the main differences between and neutropenic ulcer and an empthis ulcer? Is the red halo, the mucosa surroundingneutropenic ulcer Is pale and not red.
- The center of a neutropenic ulcer has granulation tissue with a little bit of white
What is this
Hemophilia ?
What are its types
Bleeding disorders associated with a genetic deficiency of any
one of the clotting factors
o Hemophilia A (classic type, most common type )
▪ Factor VIII deficiency
▪ X‐linked recessive
▪ Abnormal PTT
o Hemophilia B (Christmas disease)
▪ Factor IX deficiency,
▪ X‐linked recessive, Abnormal PTT
o Von Willebrand’s disease
▪ Abnormal von Willebrand’s factor, abnormal
platelets
▪ Autosomal dominant
▪ Abnormal BT, abnormal PTT
What is this clinical presentation?
o Hemophilia A
▪ A patient with only 25% of normal clotting factor VIII levels may function normally, but less than 5% will likely manifest as bruising and bleeding problems
▪ Deep hemorrhage in joints is a major complication resulting in
arthritis, ankylosis and deformity
▪ Oral findings include bleeding (often uncontrolled) upon scaling and root planing, tooth extractions and any oral lacerations
▪ Pesudotumor of hemophilia
o Tissue hemorrhage can result in a submucosal tumor‐like
mass
o Bone hemorrhage can result in intraosseous radiolucency
Hemophilia A & dental treatment
▪ Partial thromboplastin time (PTT), prothrombin time (PT), bleeding time (BT) and platelet counts, as well as consultation with physician, should be done prior to any dental treatment
▪ (PT is like INR, Normal INR between 0.8 and 1.2)
▪ Clotting factor replacement therapy (synthetic) given as indicated
▪ Avoid aspirin
What is THROMBOCYTOPENIA?
What causes it?
Markedly decreased numbers of circulating blood platelets (severe
cases < 10,000/mm³)
Etiology: Can be from:
o Decreased production (malignancy, drugs)
o Increased destruction (immunologic, drugs)
o Sequestration in the spleen (splenomegaly)
What is THROMBOCYTOPENIA?
How it presents clinically?
▪ Clinically see spontaneous gingival bleeding, petechiae,
ecchymosis and hematomas
o Clinically see some type of bleeding
What is the treatment of
THROMBOCYTOPENIA?
▪ Treatment is usually platelet transfusion
What is this clinical presentation?
o hematoma
because of THROMBOCYTOPENIA or Trauma
What is this clinical presentation?
o hematoma
because of THROMBOCYTOPENIA orTrauma
What is this clinical presentation?
Hematoma
because of THROMBOCYTOPENIA or Trauma
What is this clinical presentation?
Hematoma
because of THROMBOCYTOPENIA or Trauma
What is this clinical presentation?
Cyclic Neutropenia
Oral ulcerations on any mucosal surface exposed to minor trauma & can mimic recurrent aphthous ulcerations without the
erythematous halo that is so typical of aphthous
▪ Gingiva is most severely affected with periodontal bone loss and tooth mobility
What is this clinical presentation?
Cyclic Neutropenia
▪ Oral ulcerations on any mucosal surface exposed to minor trauma (L,T, BM and oropharynx) last 5‐7 days
▪ Depending on surface involved, can mimic recurrent aphthous
ulcerations
o Usually do not see erythematous halo that is so typical of
aphthous
▪ Gingiva is most severely affected with periodontal bone loss and tooth mobility
What is this clinical presentation?
Cyclic Neutropenia
▪ Depending on surface involved, can mimic recurrent aphthous
ulcerations
o Usually do not see erythematous halo that is so typical of
aphthous
What is this clinical presentation?
Cyclic Neutropenia
▪ Gingiva is most severely affected with periodontal bone loss and
tooth mobility
What is this clinical presentation?
Cyclic Neutropenia
o Teeth floating in air
What is this clinical presentation?
Agranulocytosis
Oral findings:
- include multiple ragged ulcerations of the oral mucosa
- o can mimic recurrent aphthous depending on sites involved
- (but often no erythematous halo) – more like neutropenic ulcer
- Gingiva is a common site due to the minor trauma caused bymastication (can resemble NUG)
What is this clinical presentation?
Agranulocytosis
Oral findings:
- include multiple ragged ulcerations of the oral mucosa
- can mimic recurrent aphthous (but often no erythematous halo)
- Gingiva is a common site due -can resemble NUG)
What is this clinical presentation?
Leukemia
Acute and chronic myelo‐monocytic leukemia are the most likely
types of leukemia to exhibit oral manifestations
o Generalized gingival hypertrophy
May create a tumor‐like mass in soft tissue
o granulocytic sarcoma/chloroma (clinically looks green)
▪ mass infiltrate into soft tissue
What is this clinical presentation?
What is this clinical presentation?
Leukemia
▪ Oral findings include gingival hyperplasia, spontaneous bleeding of gingiva, mucosal ulcerations, candidiasis, recurrent herpetic lesions, etc.
▪ Leukemic infiltrate in soft tissues (ex. gingiva) produces a diffuse,
boggy, nontender swelling that may or may not ulcerate
o most common with myelo‐monocytic types
▪ May create a tumor‐like mass in soft tissue
o granulocytic sarcoma/chloroma (clinically looks green)
▪ mass infiltrate into soft tissue
If you see children and notice they have prominent areas
of redness or red/purple and know the child has decent
oral hygiene, leukemia should be in your differential. Need
to be aware of that cause patients need to be treated in a
fairly quick fashion.
What is this clinical presentation?
Leukomia
o Leukemic infiltrate – have areas of erthema
o Looks like gingival hyperplasia, but in gingivail hyperplasia
you don’t have the same amount of redness. Leukemia has significant hyprotophy of gingivial papilla and areas that look like they have ptechia in them. With gingival
hyperplasia drug induced they are very fibrotic
What is this clinical presentation?
Leukemia
o Dilantin hyperplasia (drug induced gingival hyperplasia) –
fibrotic looking
o Has to an addition of thromocytopenia
What is this clinical presentation?
Leukemia
This is Chloroma, inflitrate gets so dense they look green. On
buccal or lingual mucosa chloroma are more of a
solitary mass, gingival it more diffuses
What is this clinical presentation?
leukomia
Cause of infiltrate they has bone loss
What is this clinical presentation?
leukomia
o Multifocal bony destruction
o Widened PDL with leukemic infiltrate
▪ Symmetric widening of PDL space in a patient that doesn’t have that normal vertical bone loss that you associate with periodontal
disease.
▪ If see symmetrical bone loss in PDL space think malignancy, neoplastic
● Lymphomas, leukemias, osteocarcoma, chondro scaroma can
do this
What is this clinical presentation?
Adult acute myeloid leukemia (AML)
is a type of cancer in which the bone marrow makes a large number of abnormal blood cells.
We see in these images:
o Hemorrhagic ulcer right vestibule
o Ulcers are deep and can go down to bone
o Ulcers resolved after treatment
What is the treatment of Leukemia?
-
Treatment depends on specific type of leukemia, but includes multi‐agent chemotherapy (often an initial high dose induction and then a lower maintenance dose)
- And for lymphoma
- Bone marrow transplant has been used with limited success
What is this clinical presentation?
Non‐Hodgkins Lymphoma (NHL)
o The most common of the lymphoproliferative diseases
o Uncontrolled proliferation of B or T cell origin derived
from a single cell (or clone)
o Typically presents with painless lymphadenopathy
(often unilateral)
▪ up to 40% are extranodal
▪ 5‐10% arise in Waldeyer’s ring
What is this clinical presentation?
Non‐Hodgkins Lymphoma (NHL)
o The most common of the lymphoproliferative diseases
o Uncontrolled proliferation of B or T cell origin derived
from a single cell (or clone)
o Typically presents with painless lymphadenopathy
(often unilateral)
▪ up to 40% are extranodal
▪ 5‐10% arise in Waldeyer’s ring
What is this clinical presentation?
Non‐Hodgkins Lymphoma (NHL)
o Typically presents with painless lymphadenopathy
(often unilateral)
▪ up to 40% are extranodal
▪ 5‐10% arise in Waldeyer’s ring
▪ Inflamatory or reactive enlarged lymph nodes tend to be
- *soft, tender, and movable**
- *▪** Lymph nodes associated with malignancy tend to be
- *hard/firm, fixed, and non‐tender**
What are the Lymphoma
Classifications?\
-
Low grade B cell (disseminated but slow growing)
- Small lymphocytic, Mantle cell, Marginal zone
- Follicular center (35‐40 % of all B cell lymphomas)
-
Moderate (aggressive)
- Diffuse B‐cell, Peripheral T‐cell
-
High Grade B cell (disseminated and rapidly progressive)
- Anaplastic large cell, lymphoblastic
- Large cell (25‐30 % of all B cell lymphomas)
- Burkitt’s
- Immunoblastic (increasingly seen in AIDS)
- As AIDS patients live longer we see this more
-
T cell lymphoma (less common than B cell, associated with palatal perforation [midline lethal granuloma]) (in differntial with deep fungal infections/candidiasis, TB, use of cocaine
- Precursor T‐lymphoblastic, T cell chronic lymphocytic,
- Hodgkin’s (bimodal ‐ late twenties and after 50 years old)
What is this clinical presentation?
Burkitt’s Lymphoma
African type (endemic)
- More common
- Peak incidence 3‐8 years of age
- Twice as common in males
- ~95% associated with the Epstein‐Barr virus
- North american or non edemic type is not associated
- with EBV
- ~88% under 3 yrs of age have jaw lesions
- Only 25% of those older than 15 do
- Typically involves:
- MD, MX, (often affects all 4 quadrants)
- Mandible or maxilla
- Abdomen
- grow quickly
What is this clinical/radiographic presentation?
Burkitt’s Lymphoma
-
North American type (sporadic)
- Typically older children (mean of 11 years)
- Involves abdomen as a mass
- uncommon in the jaws
- No association with EBV
- More localized disease
-
HIV type
- Adults
- 25% associated with EBV
- GI, marrow and CNS
Teeth floating as shown in the image
What is this clinical presentation?
Burkitt’s Lymphoma
Scattered throughout the lymphocytes are
macrophages containing nuclear debris (tingible body
macrophages)
o Creates a “starry sky” appearance
What is this clinical presentation?
T cell Lymphoma
Less common than B cell lymphomas
▪ One variant associated with palatal perforation
o Old name midline lethal granuloma
What is this clinical presentation?
Hodgkin Lymphoma
▪ Cell of origin is unknown
▪ Often begins as a unifocal disease above diaphragm and then
spreads to other sites
▪ Present with painless cervical lymphadenopathy in 60‐80% of
cases
▪ Bimodal ‐ late twenties and after 50 years old
▪ In North America and Europe, EBV positivity has been noted
in over 50 percent of mixed cellularity cases and 10 to 50
percent of nodular sclerosis cases
What is this clinical presentation?
Hodgkin Lymphoma
o Begins in lymph nodes
o 70‐75% cervical/supraclavicular
o Painless but unresolving,
o Night fevers/sweats
What is this clinical presentation?
Hodgkins lymphoma
What is this histological presentation?
Plasma Cell Disorders
Clock face nucleaus
What is this clinical presentation?
Plasma cell gingivitis
▪ Allergen causes mass infiltrate into gingival
▪ Benign
▪ Diet log to identify allergen, allergy testing
What is this clinical presentation?
Plasma cell gingivitis
▪ Allergen causes mass infiltrate into gingival
▪ Benign
Plasma cell gingivitis Treatment
▪ Diet log to identify allergen, allergy testing
▪ Ideally eliminate offending substance
▪ Can manage with topical steroids
What is this clinical presentation?
Multiple Myeloma
Monoclonal expansion of malignant plasma cell
▪ Most common in 40‐70 year old (mean 63 yo.)
▪ Present with bone pain (> 70%) and pathologic fractures
Punched out radilucency
What is this clinical presentation?
Multiple Myloma
Punched out translucency in crest
What is this clinical presentation?
Multiple myeloma
What is this clinical presentation?
Multiple Myeloma
What is this clinical presentation?
Multiple Myeloma
Pathological fracture
What is this clinical presentation?
Multiple Myeloma
Elevated M spike in serum ‐ abnormal increase in
immunoglobulin (hyperglobulinemia), most commonly IgG
▪ Reversal of normal albumin/globulin ratio
How is Multiple Myeloma treated?
Prognosis?
- Chemotherapy with or without RT
- Bone marrow transplant, interferon, antibodies made
- against tumor cells, thalidomide, bisphosphonates,
- corticosteroids, melphalan, etc.
- Even with treatment, most patients do not survive more than
- 18‐24 months
- older patients – better prognosis
- younger patients – more aggressive and worse prognosis
- IV bisphosphonate therapy puts patients at increase risk for MRONJ
What is this clinical finding?
Multiple Myeloma
Deposition of amyloid in soft tissues
o Can cause macroglossia if tongue is involved
What is this radiographical presentation?
Plasmacytoma
▪ Unifocal, monoclonal neoplastic
▪ proliferation of plasma cells
▪ 30% develop into MM over 10 yrs
▪ 50% disseminate w/in 2‐3 yrs
▪ Central Bone lesion
o Unilocular radiolucency
o Swelling or bone pain
o Non‐tender soft tissue mass
▪ 90% occur in Head and neck
