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hematopoetic part 1 Flashcards

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1
Q

Leukocytosis

A

an increase in total circulating white blood cells

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2
Q

when are these seen?

individual cell forms of leukocyotsis

A

 Neutrophilia (granulocytosis) - bacterial infections
 Lymphocytosis - viral infections
 Eosinophilia - parasitic infections, allergic reactions

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3
Q

confused with? test?

leukemoid rxn

A

an elevated white blood cell count that is a physiologic response to stress or infection
can be confused with leukemia- clarified with LAP test (low in leukemia)

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4
Q

Leukopenia

A

a decrease in total circulating white blood cell count

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5
Q

can be due to?

forms of leukopenia

A

Neutropenia – antineoplastic therapy, drugs
Lymphopenia – steroid therapy
Pancytopenia – all cell lines affected – anemia, thrombocytopenia,
neutropenia

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6
Q

interchangable terms of neutropenia

A

The terms agranulocytosis, granulocytopenia and neutropenia are
often used interchangeably

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7
Q

normal/abnormal count?

Neutropenia

A

Normal adult peripheral white blood cell count – 4,500 – 11,000 /mm3
Clinically relevant neutropenia –
Absolute Neutrophil Count (ANC) < 500 /mm3

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8
Q

neutropenia can allow sus to?

A

Susceptibility to bacterial and fungal infections

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9
Q

causes neutropenia

A

 Decreased production due to:
* Drugs
* Hematologic disease – cyclic neutropenia
* Nutritional deficiency – B12, Folate
* Myelophthisis
 Increased destruction - autoimmune reactions

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10
Q

severe neutropenia ana signs of infection

A

 In severe neutropenia the signs of infection may be absent

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11
Q

WNL WBC

A

cells/ul (4,000 – 11,000)

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12
Q

Cyclic Neutropenia

A

 Regular, periodic reductions in neutrophils

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13
Q

when are symptoms the greatest?

symptoms of cyclic neutropenia

A

 Symptoms greatest at nadir – fever, lymphadenopathy, malaise, pharyngitis, ulcerations, periodontitis

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14
Q

tx cyclic neutropenia

A

 Treatment - supportive care, cytokine
therapy (G-CSF)

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15
Q

where are the neoplastic cells

Leukemias

A

the neoplastic cells are in the bone marrow and blood

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16
Q

where are the neoplastic cells

Lymphomas

A

Lymphomas - the neoplastic cells are in the
lymph nodes – (also extranodal sites)

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17
Q

terms? describe?

Neoplasms of
Hematopoietic Cells

A

Leukemia and Lymphoma describe the tissue distribution of disease

Leukemia
* Arises in bone marrow
* Spreads to peripheral blood

Lymphoma
* Arises in peripheral lymphoid tissue, usually in lymph nodes
* Forms a discrete tissue mass
* May eventually spread to peripheral blood and bone marrow

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18
Q

onset? maturation? tx/death? cure?

Acute vs Chronic Leukemia

A
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19
Q

Classification of Leukemias by Cell of
Origin and Clinical Course

A
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20
Q

cell level? bleeding? fever? energy? what is each due to?

Clinical Symptoms of Acute Leukemia

A

Cytopenias - depression of normal bone marrow function
Bleeding – petechiae, ecchymoses, epistaxis, gingival hemorrhage due to thrombocytopenia
Fever - infections due to absence of mature granulocytes
Fatigue - anemia

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21
Q

gingiva with acute leukemia

A

Spontaneous Gingival Hemorrhage

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22
Q

what can be on palate with AL

A

Palatal Petchiae

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23
Q

what oral hemmorhage can be seen (aside from petechaie) with AL?

A

Ecchymoses

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24
Q

nose and AL

A

epitaxis (nose bleed)

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25
Acute Lymphoblastic Leukemia cells invovled? A disease of? prognosis ?
Lymphoblasts - immature precursor B or T lymphocytes arrested at early stage of development A disease of children Good prognosis with aggressive chemotherapy
26
Acute Myeloblastic Leukemia  cells? Age? Prognosis? Gingiva?
Myeloblasts – immature myeloid precursors (granulocytic, monocytic, erythroid, megakaryocytic) with no terminal myeloid differentiation Adults Prognosis – chemotherapy, bone marrow transplantation. More difficult to treat than ALL. Gingival enlargement in monocytic types of AML
27
which leukemia likely caused this
AML
28
Clinical Symptoms of Chronic Leukemia
Often clinically silent Incidental leukocytosis on CBC
29
Chronic Myelogenous Leukemia Age?  onset/progression?  chr?  signs?  crisis?  tx?
Adults Insidious onset, slow progression Philadelphia chromosome – t(9:22) bcr-abl fusion gene Splenomegaly, fever, fatigue Blast crisis Bone marrow transplantation
30
# seen with which neoplasm? Philadelphia Chromosome
SEEN WITH CML  Translocation t(9:22)  Proto-oncogene abl on long arm chromosome 9(q34)  Transposed to bcr region (breakpoint cluster region) on chromosome 22(q11)  Results in bcr-abl fusion gene  Gene product is abnormal bcr-abl tyrosine kinase  Induces cell proliferation
31
Chronic Lymphocytic Leukemia commonality?  age? symptoms?  infections? autoantibodies? Richter syndrome?
Most common type of leukemia Adults, often asymptomatic Hypogammaglobulinemia – infections Anti red cell autoantibodies – autoimmune hemolytic anemia Anti platelet autoantibodies – autoimmune thrombocytopenia Richter syndrome – may transform to high grade lymphoma
32
4 kinds of Lymphoid Neoplasms
 Lymphocytic Leukemia  Hodgkin Lymphoma  Non-Hodgkin Lymphoma  Plasma cell neoplasms
33
Clinical Presentation of Lymphoid Neoplasms: Related to ________
Related to Anatomic Distribution
34
Lymphoma presentation
Lymphoma - non-tender lymph node enlargement, extra-nodal mass
35
Leukemia cytopresentation, why?
cytopenias due to suppression of hematopoiesis
36
Plasma cell neoplasms presentation
bone pain, pathologic fracture
37
all lymphomas are?
malignant
38
classes of lymphomas
Hodgkin Lymphoma – HL Non-Hodgkin Lymphoma - NHL
39
Clinical Presentation of NHL  lymph nodes?  Generally involves?  Frequently involves?
 Painless lymphadenopathy with firm, enlarged, rubbery, freely movable, non-tender lymph nodes  Generally involves multiple lymph nodes in a non-contiguous pattern  Frequently involves extranodal sites
40
Extra-Nodal Non-Hodgkin Lymphoma Most NHL arises within? NHL may also arise? Oral mucosa?
Most NHL arises within lymph nodes – 70% NHL may also arise extra-nodally – 30% Oral mucosal NHL is extra-nodal
41
areas of mouth where extra nodal NHL may present
palate alveolar ridge
42
agression/ grades of lymphomas
All Lymphomas are Malignant Degree of aggressiveness varies Low grade - indolent, difficult to cure High grade - aggressive, often curable
43
Burkitt Lymphoma Grade?  known as  HL or NHL?  vrial association?
Burkitt Lymphoma – High Grade “African Jaw Lymphoma” B-Cell NHL EBV association
44
MALT Lymphoma Grade? Arises from?  onset?  what can increase risk?  May transform into?
MALT Lymphoma – Low Grade Arises from Mucosal- Associated Lymphoid Tissue “MALT-oma” - mature B cells Often indolent (slow onset) Salivary glands, Sjogren Syndrome can increase risk May transform to high grade lymphoma
45
Burkitt Lymphoma A sub-type of?  grade/growth rate/cell involved?  endemic predilection?
A sub-type of non-Hodgkin lymphoma (NHL) A high grade B cell neoplasm, most rapidly-growing human neoplasm The endemic form has a predilection for jaws of children
46
Burkitt Lymphoma Three clinical forms
Endemic (African) Sporadic HIV-associated
47
Cytogenetics of Burkitt Lymphoma  Translocations?  gene?  overexpression?  cellular proliferation?
 Translocations, t(8:14) is the most common  c-myc proto-oncogene on chromosome 8 has a role in cell cycle progression  Immunoglobulin gene promoters cause overexpression of c-myc  Overexpression of c-myc oncogene promotes inappropriate cellular proliferation
48
# death if untreated? Oral Findings in Burkitt Lymphoma
Rapidly growing painless swelling, producing paresthesia, loose teeth Rapid demise if untreated
49
Endemic Burkitt Lymphoma radiograph
large luceny
50
sporadic form of burkitt
51
what histological pattern can be seen with burkitts
52
# when to use? doseage/terms? chemo with burkitts
High grade lymphoma - aggressive Cures frequent with short-term, high-dose chemotherapy
53
Three Examples of Non-Hodgkin Lymphoma
Burkitt Lymphoma Diffuse large cell lymphoma MALT-oma
54
Hodgkin Lymphoma (HL) distinguished by?
The neoplastic cell: Reed-Sternberg cell (owl eyes)
55
reed sternberg cells=hogdkin lymphoma
56
The Reed-Sternberg Cell:  fraction of the tumor mass? Most are of what origin?  appearnce? hallmark for what dx?
HALLMARK CELL OF HODGKIN LYMPHOMA A minor fraction of the tumor mass Most are of B cell origin Bilobate nucleus with large inclusion-like nucleoli -“owl-eye” cell
57
General Characteristics of Hodgkin Lymphoma  age distribution?  lymph? Constitutional symptoms? Fever? Night? Weight? skin? Association with?
Bimodal age distribution - young adults, older adults Painless lymphadenopathy Constitutional symptoms variable Fever – Pel-Ebstein fever Night sweats Weight loss Generalized pruritus (itchy skin) Association with Epstein Barr virus
58
Spread of Hodgkin Lymphoma
 Uniform, predictable pattern of spread from one lymph node region to the next
59
staging of NHL
Characterizes extent of disease * Stage I– Single lymph node region * Stage II– Multiple lymph node regions – Same side of diaphram * Stage III– Multiple lymph node regions – Both sides of diaphragm * Stage IV– Disseminated disease
60
“B” Symptoms for Staging of Non-Hodgkin Lymphoma
Recurrent, unexplained fevers Night sweats Unintended weight loss
61
# low vs high grade (local/advanced) Treatment of Non-Hodgkin Lymphoma
Low-grade lymphomas - treat only if symptomatic High-grade lymphomas Localized stage - RT Advanced stage – CT or combination CT/RT
62
Staging of Hodgkin Lymphoma
Characterizes the Extent of Disease  Stage I - single lymph node region  Stage II - multiple lymph node regions on same side of diaphragm  Stage III - multiple lymph node regions on both sides of diaphragm  Stage IV - disseminated disease
63
what determines treatment of Hodgkin Lymphoma
Stage determines treatment protocol Localized (Stage I) - local radiation therapy=Risk of second cancers Disseminated (Stage IV) -chemotherapy
64
Prognosis of Hodgkin Lymphoma Stage? Histologic sub-type? Curable?
Stage - most important Histologic sub-type - least important Curable - Stage I – 90% cure rate
65
# location? spread? waldeyer? extranodal? Comparison of Hodgkin Lymphoma with Non-Hodgkin Lymphoma
66
Multiple Myeloma  age?  cells?  signs? (bones? ca?)  associated conditions? (cell levels? predespostion to?)
Older adults Disseminated neoplasm of terminally-differentiated B lymphocytes (plasma cells)  Multifocal lytic bone lesions, hypercalcemia, bone pain  Myelophthisic anemia (decreased RBC due to neoplasia in marrow), predisposition to infections
67
radiographic sign of MM
punch out lucencies of bone
68
punch out lucencies= MM
69
Oral Findings: Multiple Myeloma
Lytic lesions, loose teeth, pain, paresthesia, pathologic fracture Macroglossia due to amyloidosis
70
amyloidosis of MM can be seen where?
can be seen orally/facially
71
Laboratory Findings: Multiple Myeloma  Ca, pro, Ig  erythrocyte sedimentation rate? Rouleaux?
Laboratory Findings: Multiple Myeloma Elevated serum calcium, protein, immunoglobulins Elevated erythrocyte sedimentation rate (ESR) Rouleaux formation- stacks of RBCs
72
Ig lab finding of MM
Monoclonal gammopathy - M-spike Bence-Jones proteinuria immunoglobulin light chains
73
complications of MM
Renal failure- due to breece jame pro, toxic to kidneys Infection Anemia
74
Normal Platelet Function in Vessel Wall
Normal Platelet Function in Vessel Wall primary and secondary hemostatsis
75
bleeding time tests what cells are being assessed? represents? WNL? abnormal when? drugs that can cause abnormal result? common dx?
 Clinical assessment for adequate number and function of platelets.  The bleeding time test represents the time taken for a standardized skin puncture to stop bleeding  The normal range depends on the actual method used and varies from 2 to 9 minutes  It is abnormal when there are congenital or acquired platelet defects  Drugs – ASA, NSAIDS  Von Willebrand Disease
76
Platelet Disorders
Platelet Disorders Thrombocytopenia - decrease Thrombocytosis - increase Functional defects
77
# drugs and effects? potetnial dx? Platelet Functional Defects - Thrombasthenia
 Aspirin - inhibits aggregation for lifetime of platelet (8-10d) (irreversible)  NSAIDs - inhibits aggregation until drug eliminated (reversible)  Von Willebrand Disease - compound defect involving platelet function and coagulation pathway: Normal platelet count with increased bleeding time
78
Hemorrhage Terminology: Skin and Mucosa
 Petechiae - pinpoint hemorrhages  Purpura - petechiae become confluent  Ecchymosis - purpurae become confluent  Hematoma – cavity
79
 Petechiae  Purpura  Ecchymosis
80
Thrombocytopenia Normal platelet count? Thrombocytopenia? gradient of symtpoms
Normal platelet count - 150,000 to 450,000 /mm3 Thrombocytopenia < 100,000 /mm3
81
# production? destruction? spleen? transfusions? Causes of Thrombocytopenia
Decreased production - aplastic anemia Increased destruction – immunologic destruction Sequestration in spleen –splenomegaly Dilution - massive transfusion
82
# tx Immune Thrombocytopenic Purpura/ Idiopathic Thrombocytopenic Purpura
Autoimmune disease– antiplatelet autoantibodies = produce thrombocytopenia Treatment with steroids, splenectomy
83
ITP plattelt count
<7000/ ml
84
# forms? due to? Thrombocytosis (Thrombocythemia)
Primary thrombocytosis (essential) - hematopoietic stem cell disorder: Increased numbers of megakaryocytes producing dysfunctional platelets Reactive thrombocytosis due to: Asplenia Inflammatory disorders
85
what is the most common leukemia?
CLL

Pathology (20 decks)

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