Chapter 13: Hematologic Disorders Flashcards
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leukemia
is burkitt’s lymphoma an aggressive malignancy?
yes
the untreated patient will succomb to the disease in 6 months
platelet count less than ___/mm3 is considered severe thrombocytopenia and can result in massive bleeding and death
10,000
___ are dendritic mononuclear cells normally found in the epidermis, mucosa, lymph nodes, and bone marrow
langerhans cells
burkitt’s lymphoma has a predilection for the jaws. what specific locations are more common?
posterior jaws > anterior
maxilla > mandible
what are the neoplastic cells of hodgkin’s lymphoma?
reed-sternberg cells
typically binucleated (“owl eye” nuclei)
are males or females more commonly affected by hodgkin’s lymphoma?
males
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mycosis fungoides
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hodgkins lymphoma
reed-sternberg cells (owl-eye nuclei)
the decrease in platelets in thrombocytopenia may be due to what 3 things?
- reduced production
- increased destruction
- sequestration in the spleen
what are the two types of hodgkin’s lymphoma?
- nodular lymphocyte - predominant
- classical
what are the radiographic features of multiple myeloma?
- multiple, well-defined “punched out” radiolucencies
- may be ragged radiolucent lesions
- especially evident on skull film
what should you be able to see on a radiograph of a patient with langerhans cell histiocytosis?
- sharply demarcated (punched-out) readiolucencies
- occasionally may be ill-defined
- mandibular lesions appear scooped out because the superficial alveolar bone is destroyed
- extensive alveolar bone involvement resembles severe periodontitis clinically and radiographically the teeth look like they are floating in air
what is an example of a virus that can cause leukemia?
human T-cell leukemia/lymphoma virus type 1 (HTLV-1) transmitted by blood
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multiple myeloma
plasmacytoma typically arises in what tissue?
bone
what is the treatment for burkitt’s lymphoma?
intensive chemotherapy
with acute infections, how does lymphoid hyperplasia present?
the lymphoid hyplerplasia (tonsillar) appears as enlarged, tender, soft, freely movable nodules
what is the prognosis of langerhans cell histiocytosis?
- good if it is limited to bone
- those with visceral and cutaneous involvement have a poor prognosis
what are the oral symptoms of agranulocytosis?
necrotizing, deep, punched out ulcerations of the buccal mucosa, tongue, and palate
at what platelet count is clinical evidence of thrombocytopenia seen? this condition is often initially detected due to what?
- below 100,000/mm3
- oral lesions
non-hodgkins lymphoma is also called a ___
lymphoma
___ lesions are the most common presentation of langerhans cell histiocytosis
bone
the abnormal cells of myltiple myeloma are ___, which likely arise from ___
- monoclonal
- a single precursor that undergoes uncontrolled mitotic division
what is the treatment for anemia?
depends on correcting the underlying condition
refer to physician
what is this, and what hematologic disorder is it associated with?
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petechiae
thrombocytopenia
non-hodgkins lymphoma occurs primarily in what age patient?
adults
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mycosis fungoides
50% of patients with plasmacytoma will have multiple myeloma within ___ years
2-3
lymphoid hyperplasia may affect what tissues?
- lymph nodes
- waldeyer’s ring
- aggregates of lymphoid tissues scattered throughout the oral cavity
some patients with multiple myeloma may have pathologic ___, complain of ___, or have ___
- fractures
- fatigue
- petechiae
radiographic features of burkitt’s lymphoma are consistent with ___
a malignant process
what is the prognosis for multiple myeloma? what is the median survival?
- prognosis is poor
- median survival of 3 years
NK/T cell lymphoma is also known as ___
angiocentric T-cell lymphoma
what is the prognosis for someone with burkitt’s lymphoma?
- stage I or II - 5-year survival is 90%
- stage III or IV - 5-year survival is 80%
- due to aggressive treatment
what is the prognosis of patients with chronic myeloid leukemia?
5-year surivival is 80%, but once blast transformation occurs, the patient will usually die within 6 months
what is the treatment of multiple myeloma? does it relapse?
- chemotherapy is typically used, 60% respond
- virtually all cases relapse
- bisphosphonates are given to reduce fractures
because african burkitt’s lymphoma is seen in other areas of the world, some prefer to call it ___
endemic burkitt’s lymphoma
___ and ___ contribute to the development of leukemia
environmental and genetic factors
reduced platelet count in thrombocytopenia may e the result of what?
various causes, such as infiltration of the bone marrow by malignant cells or the toxic effects of cancer chemotherapy
___ is a unifocal, monoclonal neoplastic proliferation of plasma cells
plasmacytoma
what is the treatment for NK/T cell lymphoma?
lesions typically respond to radiation therapy
if leukemia is suspected, what should happen?
referral to appropriate physician is mandatory
hodgkin’s lymphoma is a malignant ___ disorder
lymphoproliferative
what is normal platelet count?
200,000-400,000/mm3
growth of a burkitt’s lymphoma tumor mass may cause ___ and ___
facial swelling and proptosis (protrusion of the eye)
agranulocytosis is associated with bacterial infections because ___
neutrophils are the first line of host defense
the following is an oral symptom of what hematologic disorder?
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anemia, as characterized by a bald tongue
what are the 5 histologic subtypes of the classical presentaiton of hodgkins lymphoma?
- lymphocyte rich
- nodular sclerosis - F>M; 70% of cases
- mixed cellularity
- lymphocyte depletion
- unclassifiable
what is the prognosis for patients with acute myeloid leukemia?
40% survival if they are younger than 60
10% if not
multiple myeloma is a malignancy of ___ origin
plasma cell
it has a multicentric origin within bone
plasmacytoma probably gives rise to ___
multiple myeloma
what is the average age patient with mycosis fungoides? male or female predilection?
average age is 60
M:F 2:1
in thrombocytopenia patients, the small capillaries leak blood which results in ___. if a larger quantity of blood is extravasated, a ___ will result. even larger amounts of extravasated blood results in a ___.
- petechiae (pinpoint hemorrhagic lesions)
- ecchymosis
- hematoma
what are 4 general symptoms related to the reduced oxygen-carrying capacity of the blood?
- tiredness
- headache
- fainting/feeling lightheaded
- pallor
___ is the enlargement of lymphoid tissue
lymphoid hyperplasia
what type of thrombocytopenia is a serious disorder of coagulation and is probably due to endothelial damage? you should refer to a physician for treatment; 70% survival
thrombotic thrombocytopenic purpura
which stage of mycosis fungoides is characterized by distinct papules and nodules, and has visceral involvement?
tumor
what is the treatment of lymphoid hyperplasia?
once the diagnosis is confirmed, no treatment is necessary
what is the bimodal pattern of age at diagnosis of hodgkin’s lymphoma?
- between 15-35
- after age 50
what are the histopathologic features of mycosis fungoides?
- atypical lymphocyte cells infiltrate the surface epithelium (epidermotropism is a propensity to invade the epidermis of the skin)
- these atypical lymphocytes are termed mycosis cells or sezary cells
- the atypical lymphocytes form small intraepithelial aggregates termed pautrier’s microabscesses
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langerhans cell histiocytosis
renal failure may be a presenting sign in multiple myeloma because kidneys become overburdened with excess circulating proteins, termed ___ proteins
bence jones proteins
up to 50% of multiple myeloma patients will have these proteins in their urine
what are the most common accompanying symptoms of langerhans cell histiocytosis?
pain and tenderness
what radiographic changes are seen in non-hodgkins lymphoma?
ill-defined or ragged radioluscency
langerhans cell histiocytosis lesions are found most commonly in what 4 locations?
- skull
- ribs
- vertebrae
- mandible - jaws are affected in 15% of cases
if patients with non-hodgkins lymphoma wear a denture, what might they compain of?
they may complain that their denture has become too tight
what population was burkitt’s lymphoma first documented in?
african american children
this is why it is termed african burkitt’s lymphoma
which spectrum of langerhans cell histiocytosis is characterized by the involvement of bone, skin, and viscera; more prominent involvement; mostly in infants (letterer-siwe disease)
acute disseminated histiocytosis
what is the most common type of non-hodgkin’s lymphoma of the oral cavity?
diffuse large B cell (DLBC)
clinical signs and symptoms of leukemia relate to what?
- the reduction in numbers of normal RBCs and WBCs
- decreased oxygen-carrying capacity of the blood leads to fatigue
- patients may experience easy bruising due to lack of platelets
which stage of mycosis fungoides is often mistaken for psoriasis, and is characterized by well-demarcated, scaly, itchy, erythematous plaque?
eczematous
___ leukemias run an aggressive course and will lead to the death of the patient in a few months if untreated
acute
85% of non-hodgkins lymphoma are ___ origin
B-lymphocyte
what is the treatment for mycosis fungoides?
- topical therapy is initiated first but will ultimately fail
- aggressive chemotherapy is usually necessary, especially with visceral involvement
- the disease is not curable, but is usually slowly progressive
non-hodgkins lymphoma swellings are characteristically ___
boggy
___ is a rare process that is characterized clinically by an aggressive, nonrelenting destruction of the midling structures of the palate and nasal processes
NK/T cell lymphoma
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lymphoid hyperplasia
what is the most common type of leukemia? what is the treatment?
- chronic lymphocytic leukemia, occurs in adulthood
- it is incurable, patients typically survive 10 years
what are 4 examples of leukemia?
- acute myeloid leukemia
- chronic myeloid leukemia
- acute lymphoblastic leukemia
- chronic lymphocytic leukemia
the process that leads to hodgkins lymphoma almost exclusively begins in what tissue?
- the lymph nodes, and any node is susceptible
- 75% occur in the cervical and supraclavicular nodes
___ is the general term for a decrease in volume of RBCs or in the concentration of hemoglobin
anemia
what can lymphoma of bone be mistaken for?
it can cause vague pain or discomfort that can be mistaken for a toothache
what is this, and what hematologic disorder is it associated with?
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purpura
thrombocytopenia
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NK/T cell lymphoma
aka angiocentric T-cell lymphoma
oral non-hodgkins lymphomas usually appear in either the ___ or ___
soft tissues or within the jaws
notice the mass is posterior
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burkitt’s lymphoma
this patient also had abdominal involvement
signs and symptoms of multiple myeloma result from uncontrolled proliferaiton of ___ and the uncontrolled manufacture of their ___
- tumor cells
- protein products
what chronic infections, how does lypmhoid hyperplasia present?
chronic infection (tonsillar) produce enlarged, nontender, firm, freely movable nodules
which leukemia shows specific chromosomal abnormalities?
- chronic myeloid leukemia (CML) has a translocation of the chromosomal material between the long arms of chromosomes 9 and 22
- termed the philadelphia chromosome
in hodgkins lymphoma cases, after 15 years posttreatment, patient mortality is due typically to what?
complications of therapy (secondary malignancy or cardiovascular disease)
once mycosis fungoides progresses beyond cutaneous involvement, what happens?
the patient usually dies within a year from organ failure or sepsis
what are the 4 histological types of hodgkins lymphoma?
- lymphocyte predominant (7%) - best prognosis
- nodular sclerosing (64%) - worst prognosis
- mixed cellularity (25%)
- lymphocyte depleted (4%)
non-hodgkins lymphoma lesions are typically what color?
erythematous or purple
what is the differential for a hole in the palate?
- syphilis
- NK/T cell lymphoma
- deep fungal infection
- recreational drug abuse
anemia is often a sign of what possible underlying diseases?
- renal failure
- liver disease
- chronic inflammatory conditions
- malignancies
- vitamin or mineral deficiencies
___ is the most common hematologic malignancy of african americans
multiple myeloma
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leukemia
what is the most common cutaneous lymphoma?
mycosis fungoides
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hodgkins lymphoma, characterized by a prominent supraclavicular and cervical masses
___ represents several types of malignancies of hematopoietic stem cell derivation; begins as the malignant transformation of a stem cell, which proliferates in the bone marrow and overflows into the peripheral blood
leukemia
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burkitt’s lymphoma
notice the starry sky pattern
which type of leukemia is one of the more common childhood malignancies? what percent of children survive this type of leukemia?
- acute lymphoblastic leukemia
- 80% of children survive
what do intraoral lymphoid hyperplasia lesions appear as? lesions in what location are usually bilatera?
- discrete, nontender, submucosal swellings
- may be normal in color or yellow
- if lesions appear on the posterior lateral tongue, they are usually bilateral
a significant percentage of hodgkins lymphoma is linked to which virus?
EBV
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leukemia
what is the average age of a patient with multiple myeloma? more common in males or females? african americans or caucasians?
65yo
M>W
AA>C
what is this, and what hematologic disorder is it associated with?
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hematoma
thrombocytopenia
how are bone lesions from langerhans cell histiocytosis treated?
curettage or low-dose radiation
mycosis fungoides is so-named because it resembles a fungal infection, but it is actually a ___ lymphoma
T-cell
what are some of the environmental factors which may lead to leukemia?
- exposure to pesticides and/or benzene
- ionizing radiation
without treatment, what will happen to a patient with NK/T cell lymphoma?
the patient will succumb to secondary infection, massive hemorrhage, or infiltration of vital structures
which stage of mycosis fungoides is characterized by elevated red lesions?
plaque
unlike most malignancites, hodgkin’s lymphoma neoplastic cells make up ___% of the cells in the lesion
1-3% (this is a very low number)
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langerhans cell histiocytosis
leukemias are classified according to their histogenesis and clinical behavior. what are the classifications?
- myeloid vs lymphoid
- acute vs chronic
___ leukemias run a more indolent course, though patients eventually succumb to the disease
chronic
is lymphoid tissue more prominent in older or younger patients?
younger patients, peaking around age 20, then decreasing in size
if large tonsils are symmetrical and asymptomatic, is it likely normal for that patient?
yes
tonsillar asymmetry is a potentially serious sign that should be further evaluated to rule out a metastatic tumor or lymphoma
which spectrum of langerhans cell histiocytosis is characterized by one or multiple lesions with no visceral involvement?
eosinophilic granuloma of bone
what is the average age patient with burkitt’s lymphoma? male or female predilection?
average age is 7
M>F
what is the goal of treatment of multiple myeloma?
to make the patient confortable and control the malignancy
which spectrum of langerhans cell histiocytosis is characterized by involvement of bone, skin, and viscera (hand-schuller-christian disease)?
chronic disseminated histiocytosis
mycosis fungoides progresses through 3 stages. what are they?
- eczematous
- plaque
- tumor
what is a granulocytic sarcoma?
a boggy, nontender swelling produced when leukemic cells infiltrate the oral soft tissues
increased destruction of platelets in thrombocytopenia can be due to what?
- immunologic reaction
- often precipitated by drugs - heparin is the most common
- autoimmune reaction
- component of a systemic disease, like SE or HIV
- abnormal blood clot formation, such as in patients with thrombic thrombocytopenic purpura (TTP)
most cases of agranulocytosis are induced by ___
drugs, specifically anticancer treatment
is topical therapy useful for mycosis fungoides?
it is initiated first but will ultimately fail
lymphoid hyperplasia is typically due to ___
infection
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lymphoid hyperplasia
what 2 conditions is agranulocytosis characterized by?
- neutropenia - decrease in neutrophils (<1500/mm3)
- lymphopenia - decrease in lymphocytes (immunosuppression); viral and fungal infections
over 90% of patients with burkitt’s lymphoma are positive for what virus?
EBV
with nodal presentaiton of non-hodgkins lymphoma, the patient is usually aware of a nontender mass that has been slowly enlarging for months. what happens as the malignancy progresses?
- the nodes become more numerous and are fixed
- nodes become fixed because tumor cells violate the capsule and infiltrate the adjacent tissues
american (or sporadic) burkitt’s lymphoma usually appear as a ___ mass
abdominal
chronic hyperplastic lymph nodes may be difficult to distinguish clinically from ___
lymphoma
what is the median survival time for patients with mycosis fungoides?
10 years
what are the radiographic features of burkitt’s lymphoma?
- radioluscent destruction of the bone with ragged, ill-defined margins
- may begin as several smaller sites, which eventually enlarge and coalesce
- patchy loss of the lamina dura is an early sign
what is the 5-year survival rate of patients with NK/T cell lymphoma? what about for patients with disseminated disease?
- 85%
- disseminated disease requires chemotherapy and have a 40% 5-yr survival rate
sezary syndrome (aggressive expression of mycosis fungoides) runs a fulminant course and results in the patient’s death within ___ years
3 years
___ is a hematologic disorder characterized by a markedly decreased numer of circulating blood platelets
thrombocytopenia
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plasmacytoma involving the left maxillary sinus and nasal cavity
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mycosis fungoides, characterized by Pautrier’s microabscesses
more than half of patients with langerhans cell histiocytosis are under what age?
15yo
what is the spectrum of langerhans cell histiocytosis?
- eosinophilic granuloma of bone
- acute disseminated histiocytosis
- chronic disseminated histiocytosis
if metastatic disease is excluded, multiple myeloma accounts for ___% of all malignancies that involve bone
50%
non-hodgkins lymphoma is a diverse and complex group of ___ that usually arise within ___ and grow as ___
- malignancies
- lymph nodes
- solid masses (70%)
NK/T cell lymphoma is typically found in adults or children?
adults
what are the oral symptoms of anemia?
mucosa may show pallor or the patient may have a bald tongue (tongue atropy) depending on the type of anemia, as well as burning (glossopyrosis) and pain (glossodynia) localized to the tongue
normally, what fraction of the platelet population is sequestered in the spleen? what happens to the platelets if a condition causes splenomegaly?
- 1/3
- larger numbers of platelets are taken out of circulation
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langerhans cell histiocytosis
burkitt’s lymphoma has a predilection for what location?
the jaws (up to 70% present in the jaws)
what does agranulocytosis mean?
without granulocytes (usually neutrophils)
what is numb-chin syndrome?
some patients with a non-hodgkin’s lymphoma can have parasthesia, particular with a mandibular lesion
burkitt’s lymphoma is a malignancy of ___ origin
B-cell
what type of thrombocytopenia occurs in childhood, classically after a viral infection? (90% of these cases resolve in 6 months)
idiopathic (immune) thrombocytopenia
what is the usual presenting sign of hodgkins lymphoma?
persistently enlarging, nontender, discrete mass or masses in a lymph node region
early lesions are movable, late lesions are more matted and fixed
___ is an aggressive expression of mycosis fungoides that represents dermatopathic T-cell leukemia
sezary syndrome
aggregates of lymphoid tissue are most comonly seen where?
- oropharynx
- soft palate
- lateral tongue
- floor of mouth
what are some other lesions that can occur due to thrombocytopenia?
- spontaneous gingival hemorrhage
- bleeding from minor sites of trauma
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lymphoid hyperplasia
this is not a mucocele because those are usually solitary
what is the most characteristic presenting symptom of multiple myeloma? in what location specifically?
bone pain, most common in the lumbar spine
plasmacytoma shares all the same demographics, signs, and symptoms as ___
multiple myeloma
what are the histoplathologic features of langerhans cell histiocytosis?
- identification of a lesional langerhans cell is necessary to confirm diagnosis
- ultrastructurally, langerhans cells contain rod-shaped cytoplasmic structures known as birbeck granules, which differentiate them from other APCs
___ may result in abnormal bleeding (blood can’t clot, blood leaks out of vessels, etc.)
thrombocytopenia
intraoral soft tissue non-hodgkins lymphoma lesions typically appear how? what tissues do they affect?
- nontender, diffuse swellings
- posterior hard palate, gingiva, buccal vestibule
___ is the volume of RBCs
hematocrit
what is the histopathology of burkitt’s lymphoma?
low-power magnification will show the classic “starry sky” pattern, which is due to macrophages within tumor tissues
langerhan’s cells are ___-presenting cells
antigen