Chapter 13- Hematologic disorders (neoplastic) Flashcards
What disease? Most people with _______ have too many red blood cells. But it can also cause you to have too many white blood cells and platelets.
polycythemia vera
*too many cells, but the cell are normal otherwise
Age group affected by polycythemia vera
~ 60 year old, no sex predilection
An acquired mutation of _____ is present in ___% of patients with polycythemia vera
JAK2 mutation
>95%
40% of patients have generalized pruritis w/o rash in what condition?
polycythemia vera
What is the term for a painful burning sensation, erythema, and warmth of hands and feet….Can lead to gangrene…occurs in polycythemia vera
Erythromelalgia
Patients with polycythemia vera experience _____ hemorrhage, _____ thrombus formation
increased hemorrhage
increased thrombus formation
Treatment for polycythemia vera
- phlebotomy of 500ml every other day until hematocrit is 45%
- aspirin
- megakaryocyte inhibitor
- hydroxyurea: myelosuppressive agent
___ to ___% of patients with polycythemia vera develop acute leukemia
2-10%
____% of all cancers in the US are leukemia and ____% of all cancer deaths
- 9
4. 1
Genetic disorders predisposed to leukemia (8)
- Down syndrome
- Bloom syndrome: causes problems with DNA repair, resulting in a high number of chromosome breaks and rearrangements.
- NF1
- Schwachman syndrome: malabsorption, pancreatic insufficiency, impaired bone marrow fn, skeletal abnormalities
- Ataxia-telangiectasia syndrome
- Klinefelter syndrome
- Fanconi anemia
- Wiskott-Aldrich syndrome: X-linked recessive disease characterized by eczema, thrombocytopenia (low platelet count), immune deficiency, and bloody diarrhea
genetic mutation in CML(chronic myeloid leukemia)
philadelphia chromosome
translocation 9;22
BCR-ABL fusion oncogene
precursor disease to acute myeloid leukemia
myelodysplasia syndromes
virus associated with t-cell leukemia/lymphoma
HTLV-1
human t cell leukemia/lymphoma virus 1
Prevalence of all leukemia
13:100,000
T/F Males are affected more than female with leukemia
true
AML:
Age group
median survival
broad age range including children
- 40% 5 yr survival <60 yr old
- 10% >60
CML:
Age group
median survival
3rd-4th decades
-80% 5 year survival, TKIs and BM transplant
CML: indolent period followed by blast transformation which results in death in ____
3-6 months
ALL:
Age group
median survival
Acute LymphoBlastic Leukemia
predominantly children
90% curable in children
worse prognosis in adults
CLL:
Age group
median survival
Chronic LymphoCytic Leukemia (MOST COMMON TYPE OF LEUKEMIA)
older adults
variable course 2-10+ year survival
crowding out of normal blood cells in the marrow by malignant or other proliferation
Myelophthisic anemia
Term for tumor like growths of leukemic cells
myeloid sarcoma
another name for a leukemic infiltrate in soft tissue. Called this because the tissue looks green.
chloroma
_______ is high doses of chemo agents to try to induce remission…What % of CML patients will be resistant to imatinib?
induction chemotherapy….33%
6 other names for Langerhans cell histiocytosis
histiocytosis X; langerhans cell disease; idiopathic histiocytosis; eosinophilic granuloma; langerhan cell granuloma; langerhan cell granulomatosis
Mutation in langerhan cell histiocytosis
BRAF in 40-60%
incidence of langerhan cell histiocytosis per year
1-4:1 million
Majority of cases of Langerhans cell histiocytosis occur in patients _____
younger than 15
>50%
Jaw is affected in ____% of cases of Langerhans cell histiocytosis
10-20
_______ is most affected site in oral LCH and lesions have a _______ appearance…WHAT DOES THE CEPH RADIOGRAPH OF A YOUNG LCH PT RESEMBLE?
posterior mandible scooped out **no corticated border "teeth floating in air" CEPH resembles multiple myeloma with punched out lesions on the radiograph
What are the 3 general/separate/unique clinical presentations of LCH?
- monostotic or polyostotic eosinophilic granuloma of bone
- Chronic disseminated histiocytosis (hand-schuller-christian disease)
- Acute disseminated histiocytes (letterer sewe disease)
Monostotic or polyostotic eosinophilic granuloma of bone is characterized by
1 or more bone lesions of LCH, no visceral involvement
Chronic disseminated histiocytosis: What is the HSC triad? What is the alternate name?
Bone lesions, skin, viscera HSC TRIAD (only present in few patients): 1. bone lesions, 2. exopthalmus, 3. diabetes insipidus…HAND-SCHULLER-CHRISTIAN disease
3 tissues affected in acute disseminated histiocytosis (Letterer-siwe disease- not really used)…what age group?
Cutaneous, visceral and bone marrow involvement in INFANTS
LCH is classified into ____ organ involvement or ______
single organ involvement
Multi-organ involvement
Multi-organ involvement LCH is separated into _____ dysfunction and no ____ dysfunction.
organ dysfunction
LCH with organ dysfunction is separated into low risk and high risk organ involvement
Low risk organs=
High risk organs=
low risk- skin, bone, LNs pituitary gland
High risk- lung liver spleen BM
Distinctive feature of a langerhan cell on electron microscopy? Distinctive radiographic appearance of oral cavity lesions?
Birbeck granules- rod shaped cytoplasmic structures….”teeth floating in air” on radiograph
2 IHC stains for langerhan cells…2 common histo features? (only one is in neville)
CD1a CD207(Langerin)….lots of EOs and “coffee bean” nuclei
Hodgkin lymphoma: Neoplastic cells( _____cells) only account for ____% of the cells in enlarged lymph nodes
reed-sternberg cells
0.1-2%
Hodgkin lymphoma is _____ as common as non-hodgkin lymphoma…what virus is found in a significant number of HL cases?
1/6th…EBVx`
Most common first site for Hodgkin lymphoma
Cervical supraclavicular nodes 70-75%
Hodgkin lymphoma presents in axillary and mediastinal LNs in ____%, and abdominal and inguinal nodes in _____%
5-10% each
<5%
Hodgkin lymphoma demographics
Male predilection
Bimodal age: 15-35; >50
Hodgkin lymphoma:
Category A vs. Category B
Category A= no systemic signs
Category B= systemic symptoms (weight loss, sweating, pruritis)
What is the classification system for Hodgkin Lymphoma called
Ann Arbor system
stages 1-4
What are the 2 main histological types of Hodgkin lymphoma
- Nodular lymphocyte predominant
2. Classical
What are the subtypes(5) of classical hodgkin lymphoma
- lymphocyte rich
- nodular sclerosis
- mixed cellularity
- lymphocyte depleted
- Unclassifiable
What histologic subtype of Hodgkins lymphoma supposedly is the most aggressive (even though the book says in the prognosis section that histo subtype doesn’t matter)
Lymphocyte depleted
What histologic subtype of classical Hodgkin Lymphoma is the most common
Nodular sclerosis 60-80%
What are Reed-Sternberg cells called in nodular sclerosis type classical hodgkin lymphoma
lacunar cells
If Reed-Sternberg cells are binucleated the term _____ is used. If they are multinucleated the term _____ is used.
owl-eyes
pennies on a plate
Lymphoid cells in nodular-predominant hodgkin lymphoma are called _____
popcorn cells
**these are mimicked in infectious processes like mono
____% of hodgkin lymphomas are nodular lymphocyte-predominant histologic subtype
4-5%
Current classification system for Non-hodgkin lymphoma
Modified REAL/WHO classification
____ Hodgkin cases/year
_____Non-hodgkin cases/year
9000
70,000
Increased prevalence of Non-hodgkin lymphoma in what immunologic conditions? (6)
- Bloom syndrome
- Wiskott-Aldrich syndrome
- Common variable immunodeficiency
- AIDS
- Organ transplant
- Autoimmune disease: SLE, Sjogrens, RA
HHV8 is implicated in kaposi sarcoma but also in _________
primary body cavity lymphoma and some plasmablastic lymphoma
4 Viruses associated with lymphoma
- HHV8(primary body cavity/plasmablastic)
- HTLV-1 (peripheral t cell)
- EBV(Burkitt, some Hodgkin, NK-T cell lymphoma)
- HIV
Non-hodgkin lymphoma: _____% present extranodal in the US, ~___% outside the US
20-40%
50%
What term is used for leukemic and lymphoma infiltration in the soft tissue of the oral cavity
boggy
Most common oral cavity sites for non-hodgkin lymphoma:
buccal vestibule, posterior hard palate, gingiva
Numb chin syndrome:
bony involvement of lymphoma in the mandible causing paresthesia
most common lymphoma presenting in the oral cavity
DLBCL (60%)
Another name for cutaneous T cell lymphoma
Mycosis fungiodes
Most common cutaneous lymphoma
mycosis fungoides
Prevalence of mycosis fungoides
1200/year
Mycosis fungoides demonstrates ________, a propensity to invade the epidermis of the skin
epidermotropism
Demographics of MF
M>F 2:1
middle aged
A.A. 1.5x more
3 stages of MF
- eczematous (erythematous) stage
- plaque stage
- tumor stage
Sezary Syndrome
aggressive form of mycosis fungoides
->separated from MF by the presence of cancerous lymphocytes in the blood
Mycosis cells aka Sezary cells have a ______ nucleus
cerebriform
Mycosis cells form aggregates called _____
Pautrier microabscesses
T cells of MF stain with
CD4- among other things but MF is a CD4 (t helper cell) derived lymphoma
Clinical classifications of Burkitt Lymphoma (3)
- endemic BL
- Sporadic or American BL
- Immunodeficiency-associated BL
In endemic BL more than ____% of tumor cells are positive for EBV.
90%
What is a predictor for the development of malignancy in patients with endemic type BL
levels of plasmodium falciparum (malarial organism). Higher levels= more likely to develop malignancy
% of BL cases that occur in the jaws
50-70%
Demographics associated with endemic type BL
children
central africa
male
maxilla>mandible (posterior)
Jaw involvement by endemic BL is age dependent: the older the patient the _____ likely in the jaw
less likely
90% of 3 year olds, 25% of >15 year olds
Most common clinical presentation for Sporadic/American BL
abdominal mass
EBV positivity is _______ frequent in Sporadic BL compared to endemic
less
patchy loss of lamina dura may be an early sign of?
Burkitt lymphoma
Classic histo appearance of Burkitt lymphoma
starry sky= histiocytes on a background of malignant dark blue lymphocytes
Most common translocation in Burkitt lymphoma
8;14 (q24;q32)
over expression of oncogene c-myc
Midline lethal granuloma is a non-specific name for multiple entities including ____
extranodal NK/T cell lymphoma, nasal type
-also any condition destroying the midface: infectious, wegners
According to the WHO what marker must be positive to call extranodal NK/T cell lymphoma, nasal type
EBV positivity
This was originally considered an angiocentric T cell lymphoma and is now recognized as an EBV+ B cell process with a marked T cell response
Lymphomatoid granulomatosis
what disease: Swelling of the palate → deep necrotic ulceration usually at midline.
Creation of an oronasal fistula
Secondary infection may be life threatening
Extranodal NK/T cell lymphoma, nasal type
T/F Females are more affected by Extranodal NK/Tcell lymphoma
false- males
Histo findings for EN NK/T cell lymphoma…which 3 stains can help distinguish?
angiocentric (mixed inflammatory cells around blood vessels, necrosis, large angular atypical lymphocytes)…CD56 (NKcell marker), CD3 (T cell marker), EBER (EBV-encoded RNA)
Best treatment for NK/T cell lymphoma, nasal type that is localized
Radiation 40-50Gy
similar to other T cell lymphomas
Disseminated disease is present in __% NK/T cell lymphoma, nasal type
30%
Multiple myeloma represents ___% of all malignancies
1%
What % of hematologic malignancies are multiple myeloma
10-15%
Demographics for Multiple myeloma
M>F, adults 60-70
2x more common in blacks
Most common hematologic malignancy in blacks
multiple myeloma
Characteristic presenting sign in MM
- bone pain frequently lumbar
- can also be renal failure from deposition of faulty light chains
Myelophthisic anemia
displacement of hemopoietic bone-marrow tissue by fibrosis, tumors, or granulomas
Characteristic finding in urine of 30-50% of MM patients
Bence-Jones proteins
Amyloid deposition occurs in ___% of MM patients
10-15%
intraoral: tongue
The jaw is involved in ___% of cases of multiple myeloma and can be seen radiographically as _____
30%
punched-out lesions
Protein immunoelectrophoresis in multiple myeloma should show _______
monoclonal gammopathy
What genetic finding is associated with more aggressive behavior in MM
hyperdiploid tumors
What are the 3 risk categories for multiple myeloma patients and what is the survival estimate for each
Standard risk: 6-7yrs
Intermediate
High-risk: 2-3yrs
What term is used for a plasmacytoma occurring outside of the bone
extramedullary plasmacytoma
% of extramedullary plasmacytomas ocuring in the head and neck
25%
____% of patients with a plasmacytoma show monoclonal gammopathy on evaluation of serum protein immunoelectrophoresis
25-50%
What two IHC markers are decreased in plasmacytoma in comparison to multiple myeloma lesions
cyclin D1
CD56
T/F radiation therapy is most common for plasmacytoma
true
What % of patients develop multiple myeloma after diagnosis of plasmacytoma
50% in 2-3 years
_____ prognosis for extramedullary plasmacytoma
compared to lesions of bone
Better
- Less than 30% progress to MM
- 70% disease free for 10 years after treatment
Most common type of leukemia?
Chronic lymphocytic leukemia
