Antigenic challenges in lymph nodes that lead to proliferation and enlargement known as what?
lymphoid hyperplasia
Variety of bleeding disorders associated with hereditary deficiency of any one of the coagulation factors of the blood.
Hemophilia
Accounts for 80-85% of bleeding diatheses and is result of decreased factor 8.
Severity of the disorder depends on the extent of the clotting factor deficiency.
Pts with 25% of normal factor 8 fxn normal
Pts with <5% function have significant hemorrhage d
Hemophilia A (classic hemophilia)
Bleeding disorder due to lack of factor 9.
Hemophilia B (Christmas Disease)
Most common inherited bleeding disorder due to deficiency of clotting factor that aids in platelet adhesion and helps transport and stabilize factor 8. Unusual disease as it results in both prolonged bleeding and coagulation times. Can be auto-dominant or
Von Willebrand Disease
Bleeding disorder that is X linked and therefore seen in males and carried by females. First sign of disease is usually failure of clotting after circumcision. Normal bleeding time, PT, TT, and platelet count with prolonged coagulation time and PTT.
Hemophilia A (classic hemophilia)
A rare autosomal recessive condition that results in low levels of plasminogen, the precursor to plasmin.
Plasminogen Deficiency (Ligneous Conjunctivitis).
Affected conjunctivae classically demonstrate thick, firm plaques that have been termed__________.
ligneous (wood-like).
Decreased volume of RBC's (hematocrit) or in the concentration of Hb resulting in
decreased O2 delivery to tissues
.
Usually due to underlying disease causing decreased production or increased destruction of RBC's.
Problem dx through
hematocrit, [Hg], MCV
Anemia
Codominant disorder of
Hg synthesis
.
The gene confers
resistance to malaria
and is most frequently seen in areas where the infection is endemic.
Mutation directs formation of
HbS
leading to rigid, fragile, or curved RBC's.
Sickle Cell Anemia
When only one allele is affected with sickle cell?
When both alleles affected?
1. allele- sickle cell trait
2. alleles- sickle cell disease
What 4 things occur due to sickle cell anemia? (FCWI)
1-fragile/adherent RBC's
2-Chronic hemolytic anemia
3-widespread ischemia
4-infarction and tissue death
A _____________ may occur from
hypoxia, infection, hypothermia, or dehydration
.
During attack,
severe pain
occurs from ischemia and infarction.
Sickle Cell Crisis
What is the #1 cause of death due to Sickle Cell Disease
Repeated infections of the spleen at an early age lead to increased suseptibility to infections (#1
PNEUMONIA
)
-other problems-kidney failure and stroke even at young age
What can be seen in dental radiographs that would indicate increased hematopoiesis in sickle cell anemia pts?
Hair on End skull films
Group of hereditary disorders of
hemoglobin synthesis
.
Hb is composed of 2 alpha and 2 beta globin chains. 2 genes code for B chain and 4 code alpha chain.
Thalassemia
Disease due to lack of sufficient B globin chains and accumulation of excess Alpha chains.
If 1 defective gene is inherited this is known as what?
If 2 genes are affected this is known as what?
Beta Thalassemia
-Thalassemia Minor
-Thalassemia Major (Cooley's Anemia, Mediterranean Fever)
Severe
microcytic hypochromic
anemia
is seen in...
Thalassemia Major (Cooley Anemia, Mediterranean Anemia)
Name the Hematologic Disorder:
-Massive bone marrow hyperplasia
- hepatosplenomegaly
-lymphadenopathy
-reduced trabeculation
-Chipmunk Faces
-Frontal Bossing
-Hair on End
B Thalassemia Major (Cooley Anemia, Mediterranean Anemia)
What occurs due to
jaw enlargement and frontal bossing
with
decreased trabeculation
in B thalessemia Major (2)
1. Chipmunk Facies
2. Hair on End radiographic changes
Pattern exhibits
deficient production of alpha globin chains with accumulation of excess beta
.
Alpha Thalassemia
If 1/4 alpha globin genes are altered, no clinical disease is detected.
The following versions of this disease are known as what:
2 altered genes?
3 altered genes?
4 altered genes?
-alpha thalassemia trait
-HbH disease
-Hydrops Fetalis
A-Thalassemia with:
-insignificant Anemia
-microcytosis
a-Thalassemia TRAIT
A-Thalassemia with:
-significant Hemolytic Anemia
-Splenomegaly
HbH Disease
A-Thalassemia with:
-severe generalized anemia
-fatal in first few hours of bith
-can be saved inutero with umbillical vein transfusions
- Hydrops Fetalis
Rare and dangerous disorder secondary to
failure of marrow stem cell
leading to inadequate numbers of
all types
of blood cells.
Aplastic anemia (Pancytopenia)
Immune-mediated attack of Cytotoxic T-cells targeting bone marrow stem cells - leading to failure.
Aplastic anemia (Pancytopenia)
What are some common oral signs of a patient with aplastic anemia?
Oral mucosa with petechiae, purpura, and ecchymosis, gingival hemorrhage, hyperplasia, and ulcerations due to oral infections.
Circulating neutrophils below 1500/mm3 that can also be diagnosed initially from oral manifestations.
Neutropenia
What are 6 causes of neutropenia? (MAN HBV)
1 Medications
2 Autoimmune
3 Nutritional Deficiencies
4 Hereditary disorders
5 Bacterial Infection
6 Viral Infection
Absence of granulocytic cells, particularly neutrophils.
Agranulocytosis
Disorder that typically arises a
few days following drug ingestion
and is associated with increased prevalence of
bacterial infections
Malaise, sore throat, swelling, fever, chills, bone pain, pneumonia and shock are common along with ANUG and oral ulcera
Agranulocytosis
Rare idiopathic disorder characterized by regular periodic reductions in neutrophils.
Related to a hereditary or sporadic mutation in _____________gene that triggers apoptosis of neutrophils.
Cyclic Neutropenia
neutrophil elastase gene
Cyclic Neutropenia exhibits oral ulcerations in sites of minor trauma such as :
lips, tongue, buccal mucosa, and oropharynx.
Markedly decreased number of circulating platelets (200k-400k/mm3).
Thrombocytopenia
Thrombocytopenia: increased platelet destruction which occurs due to 2 separate pathoses that trigger immune destruction of platelets.
TTP or ITP
Appears to arise from a primary endothelial disturbance that results in widespread platelet aggregation and consumption with thrombosis in the microvasculature.
-antibodies to vWF cleaving metalloprotease due triggers thrombi in small vessels and causes p
TTP- thrombotic thrombocytopenic purpura
This occurs due to development of
anti-platelet antibodies
in the absence of other causative disease processes.
Can be diagnosed only after exclusion of other known causes of thrombocytopenia.
ITP- Immune Thrombocytopenic Purpura
Rare primarily uncontrolled proliferative disorder of marrow stem cells that results in a significant increase (2-3x) in RBCs, platelets, and granulocytes. Increased blood viscosity develops with associated tendency to thrombosis formation and excess hemo
Polycythemia Vera
40% of pts with polycythemia vera report generalized pruritis without detectable skin changes. Many report burning, erythema and warmth of the hands and feet with exercise, standing, or warm temp known as what?
Erythromelalgia
Group of malignancies that arise from marrow stem cells but eventually overflows into the peripheral blood. Process is acute or chronic and is classified by the predominant cell produced by the neoplasm.
Leukemia
Leukemia that arises in childhood? Leukemia that arises in elderly adults?
ALL (acute lymphoblastic leukemia)
CLL (chronic lymphocytic leukemia)
Type of leukemia that affects a wide range that includes childhood?
Type of leukemia that exhibits its peak frequency during the 3rd and 4th decades.
AML- acute myeloid leukemia
CML- chronic myeloid leukemia
Tissue infiltration by leukemic cells may result in diffuse gingival enlargement or tumor like enlargements known as what?
Chloroma
1-Leukemia with 45% long term survival?
2-Leukemia considered incurable-avg 10yr survival?
3-Leukemia w/40% 5yr survival in pts <60
4-Leukemia with high mortality rate unless bone marrow transplantation can be performed (60% 2yr survival).
ALL
CLL
AML
CML
Ill defined and idiopathic group of disorders that exhibit a proliferation of a unique histiocyte like cell. Normally these cells present antigens to T cells. In this disease, these cells are intermixed with eosinophils, lymphocytes, plasma cells, and mul
Langerhans Cell Histiocytosis
In Langerhan Cell Histiocytosis the process can present 3 ways:
1-as solitary or multiple bone lesions known as what?
2-chronic disseminated disease known as what?
3-acute disseminated disease known as what?
1-Eosinophilic granuloma.
2-Hand-Schuller Christian Disease.
3-Letterer-Siwe disease.
What is the
diagnostic triad
of Hand Schuller Christian disease?
Bone Lesions.
(B)
Exophthalmos.
(E)
Diabetes Insipidus.
(D)
1-What is the rule on all supposed inflammatory processes of the jaws that do not respond to normal therapy in LCH?
2-This process often simulates accelerated periodontal disease that causes the teeth to appear as if what?
1-They must be investigated thoroughly.
2-Floating in Air.
A lymphoreticular malignancy composed of large unique appearing Reed Sternberg Cells, evidence suggests the neoplastic cell is of B cell origin. Cause is unknown but associated with Epstein-Barr virus infection.
Hodgkin's Disease
Diverse group of malignancies of lymphocytes that arise from within lymphoid tissue, usually nodes. Although T cell origin is seen, most lymphomas arise from B lymphocytes.
Non-Hodgkins Lymphoma
What types of patients are considered predisposed for non-hodgkins lymphoma?
Immunosuppressed
-AIDS, AI diseases, Immunosuppressive tx
What type of lymphomas do not respond well to therapy and many clinicians opt for no therapy with treatment reserved for pts with significant symptoms. Most arise in older adults and exhibit a median survival of 8-10yrs. Radiation is utilized in more aggr
Low-grade lymphomas
What type of lymphomas exhibit a 50-70% survival?
What type of lymphomas result in only 40% 5 year survival?
Intermediate lymphomas.
High-grade lymphomas.
A malignancy of T cells that exhibits a propensity to involve the epidermis; rare oral mucosal involvement does occur. The process typically arises in middle aged adults and exhibits a male predominance.
Mycosis Fungoides
Variant of mycosis fungoides that presents as an aggressive dermatopathic Tcell leukemia with widespread involvement of internal organs; this pattern exhibits a poor prognosis and is known as what?
Sezary Syndrome
Malignancy of undifferentiated B cells common in Central Africa and exhibits an association with EBV and a predilection for the jaws. Without tx most die in 6mos. With tx there is 90% response but survival time is still <1yr.
Burkitt's Lymphoma
Burkitt's lymphoma that occurs more commonly as an abdominal mass instead of jaw tumor?
Sporadic or American Burkitt's Lymphoma
Burkitts lymphoma demonstrates a cellular infiltrate that consists of dense sheets of small lymphocytic cells intermixed with benign macrophages. This is known as what?
Starry Sky
This form of Burkitts that arises in childhood with 70% having jaw involvement. Ill defined radiolucencies typically are seen in the posterior areas and exhibit a maxillary predominance.
African Burkitts
What disease is a
monoclonal malignancy of plasma cells
with a multi-centric origin within bone.
Similar unifocal bone or soft tissue proliferations are seen less commonly and are termed what?
Multiple Myeloma.
Plasmacytomas.
Serum or urine immunoelectrophoresis will demonstrate a massive overproduction of the immunoglobulin produced by the neoplastic clone of plasma cells.
Excess light chains
can be detected in the urine 30-50% of pts which can lead to renal failure. What are
Bence Jones Proteins