chpt 13- hematologic disorders Flashcards
1
Q
Proliferation of lymph cells in response to an antigenic challenge
A
Lymphoid hyperplasia
2
Q
Areas of mouth for lymphoid hyperplasia
A
oropharynx
Soft palate
Lateral tongue
Floor of mouth
3
Q
Acute vs Chronic lymphadenopathy
A
both are enlarged and freely movable, while the acute will also be soft while chronic is harder/rubbery
4
Q
Variety of bleeding disorders associated with genetic deficiencies of any one of the clotting factors
A
Hemophilia
5
Q
3 types hemophilia from most to least common
A
Hemophilia A
Hemophilia B
von Willebrand’s Disease
6
Q
Hempophillia A is what factor deficiency
A
VIII
7
Q
Hemophilia B is what factor deficiency
A
IX
8
Q
von Willebrand’s disease is what deficiency
A
plasma glycoprotein
9
Q
Hemophilia A is what type of genetic disorder and who is it expressed in
A
X-linked disorder with female carrier, but expressed in males
10
Q
General term for a drop in the hematocrit or hemoglobin concentration
A
Anemia
11
Q
general causes of anemia
A
blood loss
Decreased production of erythrocytes Increased destruction of erythrocytes
12
Q
Anemia develops when…
A
not enough red blood cells(RBC) to transport oxygen to organs or RBC deficient in hemoglobin
13
Q
What is the treatment of Hemophilia (I know this is out of place)
A
Replacement therapy with appropriate clotting factor
14
Q
General symptoms of Anemia
A
tiredness, headache, lightheadedness (think of anything that will happen with low oxygen)
15
Q
*****genetic disorder of hemoglobin synthesis that results from thymine substituting for adenine and eventually causing a rigid-curved(sickle) shaped cell instead of the normal biconcave shape
A
Sickle Cell Anemia
16
Q
What tongue morphology is associated with vitamin deficiency and anemia
A
Bald tongue
17
Q
Sickle Cell Anemia trait common in what demographic
A
Black and Mediterranean origin
18
Q
Symptoms of sickle cell crisis
A
extreme pain due to infarction of tissue
19
Q
Skull radiograph character of Sickle Cell
A
hair on end due to more marrow for extra hematopoiesis
20
Q
2 disorders that cause Hair on End pattern
A
- Sickle cell anemia
- Thalassemia
21
Q
Sickle Cell anemia is prone to what bacterial infection
A
S. Pneumonia
22
Q
Character of sickle cells
A
cannot pass through small blood vessels. Destroyed quicker than normal blood vessels
23
Q
5 Things that can trigger sickling phenomenon
A
exercise Exertion Admin of general anesthetic Pregnancy Sleep
24
Q
Treatment of Sickle Cell Anemia
A
symptomatic and supportive therapy, oxygen, rest, fluids
25
Hemoglobin synthesis disorder characterized by reduced synthesis of either the alpha-globin or beta- globin chains of the hemoglobin molecule
Thalassemia
26
Homozygoud Thalasemia is major or minor and what does it cause
Major. Hemolytic anemia due to RBC membrane damage and destruction of RBC’s
27
Clinical look of Thalasemia major patient
*****prominent cheekbones, depressed nasal bridge, prominent premaxilla, flaring of maxillary anterior teeth
28
Key trait on radiograph to associate with Thalassemia
Thinning of Lamina dura
29
Thalassemia treatment
- blood transfusions and deferoxamine infusions nightly.
| - Splenectomy
30
****Rare, life-threatening anemia caused by the failure of hematopoietic prescursor cells in bone marrow to produce adequate numbers of all types of blood cells
Aplastic Anemia
31
Aplastic anemia cause of death
White Blood Cell (WBC) deficiency (neutropenia, leucopenia, granulocytopenia) leads to bacterial and fungal infections
32
Granulocyte, platelet, reticulocyte numbers to diagnose Aplastic Anemia
granulocytes <20,000
| Normal Granulocytes/neutrophiles = 6,000-10,000
33
Bone marrow character of aplastic anemia
relatively acellular with extensive fatty infiltration
34
Best treatment for Aplastic Anemia
patients younger than 40 with HLA matched bone marrow donor
35
***Decrease in the number of circulating neutrophils below 1500/mm3
Neutropenia
36
2 mechanisms that cause neutropenia
decreased production
| Increased destruction
37
What nutritional deficiency can cause neutropenia
*****Vit B12 or folate deficiency
38
Bacteria susceptible to with neutropenia
S. aureus and gram negatives
39
Oral character of neutorpenia
gingival ulcers lacking characteristic erythmatous halo
40
****Neutropenia treatment
Cytokine granulocyte colony stimulating factor
41
****Condition where the cells of the granulocytic series, particularly neutrophils, are absent, through either decreased production or increased destruction
Agranulocytosis
42
Oral lesions common with Agranulocytosis
****Ulcerations and Necrotizing Ulcerative Ginigivitis (NUG)
43
****Agranulocytosis treatment
if suspect drug-induced, stop drugs. Give Granulocyte-macrophage colony stimulating factor (GM-CSF)
44
****Rare idiopathic hematological disorder that is characterized by regular periodic reductions in neutrophil population
cyclic neutropenia
45
Normal cycle for cyclic neutropenia
approximately 21 day cycle
46
Symptoms of cyclic neutropenia
seem to have flu every 3 weeks dor 3-6 days
47
character of ulcers in cyclic neutropenia
****have erythmatous halo
48
****Decreased number of circulating blood platelets due to either decreased production, increased destruction or sequestration in the spleen
Thrombocytopenia
49
Normal platelet count
200,000 – 400,000/ mm3
50
Platelets required for what
hemostasis and clot formation
51
Things that can cause reduced platelet production
Malignancy of bone marrow
| Toxic effects of cancer chemotherapy
52
2 systemic diseases that can cause thrombocytopenia
Systemic lupus (SLE) and HIV
53
At what platelet count is thrombocytopenia seen
<100,000/mm3
54
Thrombocytopenia can cause what lesions
****petechia, ecchymosis, hematoma
55
Clinical signs of thrombocytopenia
-epistaxis (nose bleeds)
-Hemoptosis (vomiting blood = pulmonary
hemorrhage)
56
****Thrombocytopenia occurring in childhood after a nonspecific viral infection with quick and sever onset but can self resolve in 4-6 weeks
Idiopathic (immune) Thrombocytopenic purpura (ITP)
57
****Serious disorder of coagulation thought to be caused by endothelial damage triggering formation of numerous thrombi within small blood vessels of body
Thrombotic Thrombocytopenic purpura (TTP)
58
Thrombocytopenia treatment
discontinue offending drug and platelets return in a few days
59
Idiopathic thrombocytopenic purpura treatment
spontaneous resolution or give corticosteroids or IV immunoglobulin
60
Thrombotic thrombocytopenic purpura (TTP) treatment
plasma pheresis or plasma exchange. Still have guarded prognosis, but no longer certain death
61
****Idiopathic hemorrhagic disorder caused by increase in mass of Red Blood Cells
Polycythemia Vera
62
Peripheral vascular event affect hands and feet of pt with Polycythemia Vera where there is a painful burning sensation accompanied by erythema and warmth that may eventually lead to thrombotic occlusion of vessels supplying digits
Erythromelalgia
63
Gen mutation in 95% of Polycythemia Vera
Tyrosine kinase gene mutation
64
40% of Polycythemia Vera patients complain of this after a bath
****itching with absence of rash
65
****3 treatments of Polycythemia Vera
1) Phlebotomy (500 ml blood drain/day)
2) Anagrelide HCl to control platelet levels
3) Antihistamines for purities (itching)
66
Term for several types of malignancies of hematopoietic stem cell derivation
Leukemia
67
Syndromes with increased risk of Leukemia
- Downs
- Neurofibromatosis 1
- Klinefelter’s (XXY)
- Fanconi’s Anemia
68
Which leukemia affects children and adults
Acute Myeloid leukemia
69
Which leukemia peaks in the third and fourth decade
Chronic myeloid
70
Which leukemia is almost always in children
Acute lymphoblastic leukemia
71
Which leukemia primarily affects elderly adults
Chronic Lymphocytic Leukemia
72
malignant proliferation in bone marrow with leukemia causes what anemia
myelophthisic anemia
73
As marrow is crowded out in leukemia what happens to RBC
reduced oxygen capacity = fatigue
74
When platelets drop below 20,000 mm3 (thrombocytopenia induced by leukemia ) what is seen on the posterior hard and soft palates
petechia
75
Leukemia gingival
****boggy gums
76
6 treatments for Leukemia
1) drugs + radiation
2) Bone marrow transplant nt <45
3) Platelet transfusion
4) Packed Red blood cells for anemia
5) Antibiotics for infection
6) Optimal oral hygiene
77
4 types of Leukemias and their age ranges
- Acute Myeloid Leukemia (adults & children)
- Chronic Myeloid Leukemia (30-40 year olds)
- Acute Lymphoblastic Leukemia (children)
- Chronic Lymphocytic Leukemia (older adults)
78
Most common Leukemia and is it considered curable
Chronic Lymphocytic Leukemia. Not curable
79
Cause of death in Chronic Myeloid Leukemia
Blast transformation after long indolent period
80
Recognize histology slide with large white cells indicating leukemia beside a picture of someone with inflamed gingiva
leukemia
81
Dendritic mononuclear cells found in the epidermis, mucosa, lymph nodes, and bone marrow acting as antigen presenting cells to T-cells
Langerhans cells
82
*****Spectrum of disorders characterized by a proliferation of histiocyte-like (Langerhans cells) cells that are accompanied by varying numbers of eosinophils, lymphocytes, plasma cells, and multinucleated giant cells. Shows monoclonal proliferation as would be seen in neoplastic process
Langerhans cell histiocytosis
83
3 types of Langerhans Cell Histiocytosis
-Monostotic or polyostotic Eosinophilic Granuloma
-Hand Schuller Christian Disease/ Chronic
Disseminiated histiocytosis
-Letterer-Siwe Disease/Acute Disseminated
histiocytosis
84
What is the most common Langerhans Cell histiocytosis
Hand Schuller Christian Disease/ Chronic Disseminiated histiocytosis
85
****Most common clinical presentation of Langerhans Cell Histiocytosis
Bone lesions
86
Radiographic appearance of Langerhans Cell histiocytosis in jaw
*****Tooth floating in air appearance
87
****Malignant lymphoproliferative disorder usually beginning with lymph nodes presenting as persistently enlarging, nontender mass or masses in one lymph node region
Hodgkin’s Lymphoma
88
Characteristic cells of Hodgkin’s lymphoma
****Reed-Sternberg cells (owl eyes)
89
Hodgkin’s lymphoma found 75 % in what lymphnodes
Cervical and supraclavicular nodes
90
Hodgkin’s lymphoma treatment
based on staging. Stage I & II use mild radiation and multiagent chemo. Stage III & IV use chemo
91
Most common histologic type of Hodgkin’s lymphoma
Nodular Sclerosis (frequent in 20 year old females)
92
****Group of malignancies of lymphoreticular histogenesis arising in the lymph nodes and growing in a solid mass
Non-Hodgkin’s lymphoma
93
Non-Hodgkin’s lymphoma 85% arise from what lymphocyte
****B Cells
94
3 categories of Non-Hodgkin’s lymphoma
Low grade
Median grade
High grade
95
Non-Hodgkin’s Lymphoma in bone character
will be ill defined/ ragged radiolucency w/ expansion & cortical perforation
96
Non-Hodgkin’s lymphoma oral common sites
Buccal vestibule, posterior hard palate, gingival
97
What can Non-Hodgkin’s lymphoma in jaw bone be mistaken for
****toothache
98
Numb chin syndrome in Non-Hodgkin’s lymphoma why
mandibular lesion hitting IAN
99
Non-Hodgkin’s lymphoma treatment
all with radiation &/or chemo. Low grade recurs, Intermediate and High grade responds, but low cure rate
100
CD 4 T cell lymphoma, but sounds like a fungal infection
Mycosis Fungoides
101
Demographics for Mycosis Fungoides
2:1 male, 55-60
102
3 stages of Mycosis Fungoides
Eczematous, plaque, tumor
103
Which stage of Mycosis Fungoides can be confused with psoriasis
eczematous
104
Aggressive expression of T Cell Leukemia
Sezary syndrome
105
Cure rate of Mycosis Fungoides
not curable, but usually slowly progressive
106
****Malignancy of B-lymphocyte origin that represents an undifferentiated lymphoma
Burkitt’s Lymphoma
107
Burkitt’s Lymphoma is related pathogenetically to what virus
****Epstein Barr Virus
108
Demographics and location of Burkitt’s Lymphoma
Children ~ 7 yrs, 50-70% in posterior jaws, Maxilla>Mandible
109
Jaw involvement of Burkitt’s Lymphoma is based on what
age. 3 yrs old, 90% have jaw lesion vs. >15 years old, 25% have jaw lesion
110
Radiographic character of Burkitt’s lymphoma
****ragged borders, patchy loss of lamina dura
111
Histo pattern of Burkitt’s Lymphoma
****Starry sky
112
Burkitt’s lymphoma treatment
intense chemo with cyclophosphamide w/ 90% disease free. Death w/in 4-6 months if untreated
113
****Malignancy of plasma cell origin that often appears to have a multicentric origin within bone
Multiple Myeloma
114
Demographics and symptoms of Multiple Myeloma
40 year old black males w/ bone pain
115
Radiographic appearance of Multiple myeloma and jaw incidence
****“punched out” boney lesions. 30% jaw
116
other common presenting symptom beyond bone pain of Multiple Myeloma and the urine protein used to diagnose
Renal failure due to kidney overburden w/ light chains. Bence Jones Proteins = circulating light chains
117
Multiple Myeloma tongue character due to amyloid
enlarged, firm, nodular
118
Histo of Multiple Myeloma
malignant plasma cells w/ eccentric nuclei and stippled nuclear chromatin in sheets indicating monoclonal origin
119
Treatment Multiple Myeloma
Aggressive chemo and bone marrow transplant if able. 25% 5 year survival if chemo only. 50% survival if chemo and bone marrow transplant
120
****Unifocal, monoclonal, neoplastic proliferation of plasma cells in bone (versus the multcentric origin of Multiple Myeloma)
Plasmacytoma
121
Demographics for Extramedullary Plasmactyoma
6:1 Males, 90% head and neck
122
Most common site for Plasmacytoma
****Spine
123
What may Plasmacytoma progess to
Multiple Myeloma
124
Treatment of Plasmacytoma
radiation at least 4000 cGy
125
Which has better prognosis Plasmacytoma or Extramedullary Plasmacytoma
Extramedullary plasmacytosis, 70% 10 year disease free w/ 30% convert to multiple myeloma
126
Radiographically, is the plasmacytoma a single or multiple punched out bony lesions
****Single punched out boney lesion
