White Blood cells and Platelets Pathology Flashcards
what is leukocytosis
an increase in total circulating white blood cells
what is neutrophilia (granulocytosis) seen in
bacterial infections
what is lymphocytosis seen in
viral infections
what is eosinophilia seen in
parasitic infections, allergic reactions
what is leukemoid reaction
an elevated white blood cell count that is a physiologic response to stress or infection
what is leukopenia
a decrease in total circulating white blood cell count
what is neutropenia caused by
antineoplastic therapy, drugs
what is lymphopenia caused by
steroid therapy
what is pancytopenia
all cell lines are affected- anemia, thrombocytopenia, neutropenia
what terms are used interchaneably with granulocytopenia
neutropenia and agranulocytosis
describe neutropenia
- normal adult peripheral white blood cell count - 4,500- 11,000 / mm^3
- clinically relevant neutropenia- absoulte neutrophil count less than 500/mm^3
- susceptibility to bacterial and fungal infections
what are the causes of neutropenia
- decreased production
- increased destruction- autoimmune reactions
- in severe neutropenia the signs of infection may be absent
decreased production is caused by what in causes of neutropenia
- drugs
- hematologic disease- cyclic neutropenia
- nutritional deficiency- B12, folate
- melophthisis
what is the treatment of neutropenia
clindamycin 1200 mg/day
what are the clinical lab values for neutropenia
- WBC: 1,600 cells/ul
- polys: 4%
- lymphocytes: 69%
- monocytes: 27%
- eosinophils: 0%
- basophils: 0%
- absolute neutrophil count = 64 neutrophils/ul
describe cyclic neutropenia- symptoms and tx
- regular, periodic reductions in neutrophils
- symtpoms greatest at nadir- fever, lymphadenopathy, malaise, pharyngitis, ulcerations, periodontitis
- treatment- supportive care, cytokine therapy
what are leukemias
the neoplastic cells are in the bone marrow and blood
what are lymphoams
the neoplastic cells are in the lymph nodes- also extranodal sites
what are the neoplasms of hematopoietic cells
leukemia and lymphomas
describe leukemia
- arises in bone marrow
- spreads to peripheral blood
describe lymphomas
- arises in peripheral lymphoid tissue, usually in lymph nodes
- forms a discrete tissue mass
- may eventually spread to peripheral blood and bone marrow
describe acute leukemia
- abrupt, stormy onset
- no maturation- precursor cells proliferate
- kills rapidly without treatment
- cure is possible
describe chronic leukemia
- insidious course
- maturation - mature cells proliferate
- often not treated unless symptomatic
- cannot be cured
describe white blood cell maturation
- pluripotent stem cell -> myeloid stem cell or lymphoid stem cell
- myeloid stem cell -> erythroblast, megakaryoblast, myeloblast, monoblast
- lymphoid stem cell-> pre B lymphocyte or pro T lymphocytes
what are the classifications of leukemias by cell of origin and clinical course
- acute lymphoblastic leukemia
- acute myelogenous leukemia
- chronic lymphoblastic leukemia
- chronic myelogenous leeukemia
what are the clinical symptoms of acute leukemia and describe each
- cytopenias- depression of normal bone marrow function
- bleeding- petechiae, ecchymoses, epistaxis, gingival hemorhhage due to thrombocytopenia
- fever- infections due to absence of mature granulocytes
- fatigue- anemia
describe acute lymphoblastic leukemia
- lymphoblasts - immature precursor B or T lymphocytes arrested at early stage of development
- a disease of children
- good prognosis with aggressive chemotherapy
describe acute myeloblastic leukemia
- myeloblasts - immature myeloid precursors with no terminal myeloid differentiation
- adults
- prognosis- chemotherapy, bone marrow transplantation, more difficult to treat than ALL
- gingival enlargment in monocytic types of AML
what are the types of AML
- granulocytic
- monocytic
- erythroid
- megakaryocytic
what are clinical symptoms of chronic leukemia
- often clinically silent
- incidental leukocytosis on CBC
describe chronic myelogenous leukemia
- adults
- insidious onset, slow progression
- philadelphia chromosome- t(9:22) bcr- abl fusion gene
- splenomegaly, fever, fatigue
- blast crisis
- bone marrow transplantation
describe the philadelphia chromosome
- 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
describe chronic lymphocytic leukemia
- most common type of leukemia
- adults are often asymptomatic
- hypogammaglobulinemia- infections
- anti red cell autoantibodies- autoimmune hemolytic anemia
- anti platelet autoantibodies- autoimmmune thrombocytopenia
- richter syndrome- may transform to high grade lymphoma
whaat are the lymphoid neoplasms
- lymphocytic leukemia
- hodgkin lymphoma
- non-hodgkin lymphoma
- plasma cell neoplasma
what is the clinical presentation of lymphoid neoplasms with lymphomas
non tender lymph node enlargement, extra nodal mass
what is the clinical presentation of lymhoid neoplasms with leukemias
cytopenias due to suppression of hematopoiesis
what is the clinical presentation of plasma cell neoplasms
bone pain, pathologic fracture
what are the malignant lymphomas
- hodgkin lymphoma
- non hodgkin lymphoma
what is the clinical presentation of non hodgkins lymphoma
- painless lymphadenopathy with firm, enlarged, rubbery, freely movable, non tender lymph nodes
- generally involves multiple lymph nodes in a non- contuguous pattern
- frequently involves extranodal sites
what is the prevalence of NHL arising in lymph nodes? extra nodally?
- lymph nodes: 70%
- extra- nodally: 30%
oral mucosa NHL is_____
extra nodal
all lymphomas are ____
malignant
what are the variations of lymphomas
- degree of aggressiveness varies
- low grade- indolent, difficult to cure
- high grade- aggressive, often curable
describe burkitt lymphoma
- high grade
- “african jaw lymphoma”
- B-cell NHL
- EBV association
- sub type of NHL
- a high grade b cell neoplasm, most rapidly growing neoplasm
- endemic form has predilection for jaws of children
describe MALT lymphomas
-low grade
- arises from mucosal - associated lymphoid tissue - “MALT- oma” - mature B cells
- often indolent
- salivary glands, Sjogren syndrome
- may transform to high grade lymphoma
what are the 3 clinical forms of burkitt lymphomas
- endemic (african)
- sporadic
- HIV associated
what is the association of epstein- barr virus with human disease
- infectious mononucleosis
- lymphomas- NHL and HL
- nasopharyngeal carcinoma
- oral hairy leukoplakia
what are the cytogenetics of burkitt lymphoma
- 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
what are the oral findings in burkitt lymphoma
- rapidly growing painless swelling, producing paresthesia, loose teeth
- rapid demise if untreated
describe chemotherapy in burkitt lymphoma
-high grade lymphoma- aggressive
- cures frequent with short term, high dose chemotherapy
what are the three examples of NHL
- burkitt lymphoma
- diffuse large cell lymphoma
- MALT-oma
what is the neoplastic cell in HL
Reed- Sternberg cell
describe the Reed- Sternberg cell
- a minor fraction of the tumor mass
- most are of B cell origin
- bilobate nucleus with large inclusion like nucleoli- “owl eye” cell
what are the general characteristics of hodgkin lymphoma
- bimodal age distribution- young adults and older adults
- painless lymphadenopathy
- constitutional symptoms variable- fever - Pel- Ebstein fever, night sweats, weight loss, generalized pruritus
- association with EBV
describe the spread of hodgkin lymphoma
- uniform, predictable pattern of spread from one lymph node region to the next
what is the staging of NHL
- stage I: single lymph node region
- stage II: multiple lymph node regions, same side of diaphragm
- stage III: multiple lymph node regions, both sides of diaphragm
- stage IV: disseminated disease
what are the “B” symptoms for staging of NHL
- recurrent, unexplained fevers
- night sweats
- unintended weight loss
what is the treatment of NHL
- low grade lymphomas- treat only if symptomatic
- high grade lymphomas: localized stage - RT. advanced stage- CT or combination CT/RT
what is the tx of HL
- stage determines tx protocol
- localized (stage I) - local radiation therapy
- disseminated (Stage IV) - chemotherapy
- risk of second cancers
what is the prognosis of HL
- stage- most important
- histologic sub type- least important
- curable- stage I- 90% cure rate
what is the location of HL? NHL
-HL: single axial group of nodes
- NHL: multiple peripheral nodes
what is the spread of HL? NHL?
- HL: predictable- orderly contiguous spread
- NHL: unpredictable- con contiguous spread
does HL involve Waldeyer ring? NHL?
- HL: rarely
- NHL: commonly
what is the prevalence of extra nodal in HL? NHL?
- HL: rare
- NHL: common
describe multiple myeloma
- older adults
- disseminated neoplasm of terminally differentiated B lymphocytes
- multifocal lytic bone lesions, hypercalcemia, bone pain
- myelophthisic anemia, predisposition to infections
what are the oral findings of multiple myeloma
- lytic lesions, loose teeth, pain, paresthesia, pathologic fracture
- macroglossia- amyloidosis
what are the lab findings for multiple myeloma
- elevated serum calcium, protein, immunoglobulins
- elevated erythrocyte sedimentation rate (ESR)
- rouleaux formation
- monoclonal gammopathy - M- spike
- Bence- Jones proteinuria- immunoglobulin light chains
what are the complications with multiple myeloma
- renal failure
- infection
- anemia
what is primary hemostasis
platelet plug
what is secondary hemostasis
fibrin clot
what is the bleeding time test
- clinical assessment for adequate number and function of platelets
- represents the time taken for a standardized skin puncture to stop bleeding
what does the normal range of the bleeding time test depend on
the actual method used and varies from 2-9 minutes
when is the bleeding time test abnormal
- when there are congenital or acquired plateley defects
- drugs- ASA, NSAIDS
- VW disease
what are the platelet disorders
- thrombocytopenia- decrease
- thrombocytosis- increase
- functional defects
what is thrombasthenia caused by
- aspirin- inhibits aggregation for lifetime of platelet - 8-10 days - irreversible
- NSAIDs- inhibit aggregation until drug eliminated - reversible
- von willebrand disease - compound defect involving platelet function and coagulation pathway
- normal platelet count with increased bleeding time
what are petechiae
pinpoint hemorrhages
what are purpura
petechiae become confluent
what are ecchymosis
- purpurae become confluent
what is a hematoma
cavity
what are the lab values for thrombocytopenia
- normal platelet count -150,000 - 450,000 / mm^3
- thrombocytopenia less than 100,000 / mm^3
what are the causes of thrombocytopenia
- decreased production - aplastic anemia
- increased destruction - immunologic destruction
- sequestration in spleen - splenomegaly
- dilution - massive transfusion
describe immune thrombocytopenic purpura and tx
- autoimmune disease- antiplatelet autoantibodies produce thrombocytopenia
- treatment with steroids, splenectomy
what is the platelet count with immune thrombocytopenic purpura
7,000/ ml
describe primary thrombocytosis
- essential
- hematopoietic stem cell disorder
- increased number ofs of megakaryocytes producing dysfunctional platelets
what is reactive thrombocytosis
- asplenia
- inflammatory disorders
