Chapter 13 - White Blood Cells Flashcards
(159 cards)
0
Q
Neutropenia
A
Decrease in neutrophils
1
Q
What is leukoerythroblastosis
A
Abnormal release if immature precursors into the peripheral blood due to disease
2
Q
Agranulocytosis
A
Clinically significant reduction in neutrophils
3
Q
How does agranulocytosis occur? (2)
A
Ineffective granulopoeisis (decreased production) or accelerated removal of neutrophils (increased destruction)
4
Q
Most common cause of agranulocytosis
A
Drug toxicity (alkylating agents, chloramphenicol, sulfonamides, chlorpromazine, thiouracil)
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Q
What is a consequence of agranulocytosis
A
Infections, especially lesions of the oral cavity
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Q
Leukocytosis
A
Increase in number of white cells in blood
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Q
What is a leukmoid reaction
A
In severe infections, immature granulocytes appear in the blood simulating a myeloid leukemia
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Q
Causes of neutrophils leukocytosis (3)
A
Infection, sterile inflammation (ie tissue necrosis like MI), drugs (agranulocytosis)
9
Q
Causes of eosinophilic leukocytosis (2)
A
Allergic reactions, parasitic infections
10
Q
What causes basophilic leukocytosis
A
Myeloproliferative disorders
11
Q
What causes lymphocytosis
A
Viral infections (cmv, mono), bacterial infections (bordatella)
12
Q
What causes monocytosis
A
Chronic infections (tb), collagen vascular diseases, inflammatory bowel diseases
13
Q
Pathophysiology of lymphadenitis
A
Activation of immune cells –> primary follicles enlarge into germinal centers –> paracortical t-cell zones undergo inflammation
14
Q
What are the nodes in acute nonspecific lymphadenitis like
A
Enlarged and painful, may have draining sinuses
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Q
Causes of acute lymphadenitis and what nodes are most likely involved
A
Systemic diseases like mono, cervical and inguinal
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Q
Causes of chronic lymphadenitis
A
Cancer
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Q
What are the nodes like in chronic lymphadenopathy
A
Nontender since the growth occurs slowly over time
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Q
What nodes are most likely involved in chronic lymphadenitis
A
Inguinal and axillary
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Q
Most lymphoid neoplasms are of what origin
A
B-cell
20
Q
What is the most common cancer of children
A
Acute lymphoblastic leukemia
21
Q
What are some positive markers for pre b-cell acute lymphoblastic leukemia. What is expressed in the more mature forms
A
Deoxynucleotidal transferase (TdT) and common ALL antigen (CALLA, CD10). CD 19 and CD 20
22
Q
What translocation is for B-cell ALL and what kind of prognosis does it have
A
t(12;21) –> good prognosis
t(9;22) –> has a poor prognosis because there is mutation in the Philadelphia chromosome (seen with adults)
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Q
What positive markers are there for t-cell ALL
A
TdT and negative for CD10, CD2-8
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What metastatic complications are associated with B-cell ALL
testicular enlargement, abrupt onset, anemic symptoms, CNS manifestations
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What metastatic complications are associated with T-cell ALL
Mediastinal mass, abrupt onset, anemic symptoms, CNS manifestations
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Does ALL in pediatric patients have a good prognosis?
Yes 95% complete emission
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What does the bone marrow look like in ALL
Crowded with lymphoblasts
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What disease is ALL associated with
Down's syndrome
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What is the most common leukemia in the Western World
Chronic lymphocytic leukemia
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In CLL what does peripheral show
Smudge cells (small round lymphocytes with scant cytoplasm that get distrusted while trying to make a smear
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In CLL if you get lymph node involvement and generalized lympohadenopathy what will this be called
Small lymphocytic lymphoma, stays localized
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Complications of CLL
Hypogammaglobinemia (leads to infection) , autoimmune hemolytic anemia, richter syndrome
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What is Richter syndrome
CLL/SLL transforms into a diffuse large B-cell lymphoma with a rapidly enlarging mass within a lymph node or spleen
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Age group for CLL
Greater than 60 years old
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Follicular lymphomas arise from what ? And are associated with what gene defect
From germinal B cells and chromosomal translocation of IgH on chromosome 14 and Bcl2 (antagonizes apoptosis) on chromosome 18
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A positive marker that might be seen with follicular lymphoma
CD20
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What may a follicular lymphoma progress to?
Diffuse large cell B lymphoma
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What is the most common form of non Hodgkin's lymphoma and is it aggressive or benign?
Diffuse large b-cell lymphoma, highly aggressive
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What age group does diffuse large b-cell lymphoma typically present in
60 years
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How does diffuse large b-cell lymphoma arise?
Derives from a germinal center or transformation from a low grade lymphoma
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How does a diffuse large b-cell lymphoma clinically present?
Presents as a rapidly enlarging mass at a nodal or extra nodal site
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Diffuse large b-cell lymphomas can be complicated by
HIV either immunodeficiency related with EBV or an effusion lymphoma with malignant pleural or ascitic effusion (with HHV-8)
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Burkitt's lymphoma is associated with what disease
EBV
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What types of Burkitt's lymphoma are there and what organs do they involve
African type (jaw) and American type (abdomen)
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What appearance of Burkitt's lymphoma have on histology and what causes it
Starry sky appearance from clear cytoplasmed macrophages that ingested nuclear remnants from tumor cells
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What is the gene mutation associated with Burkitt's lymphoma
c-MYC on chromosome 8 to the Ig heavy chain locus on chromosome 14
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What markers does CLL/SLL Express
CD 5 and 20
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What are plasma cell dyscrasias
Bcell proliferations with neoplastic plasma cells that secrete immunoglobin or a immunoglobin fragment
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What do neoplastic plasma cells synthesize an excess of
Light or heavy chains with complete immunoglobins
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What body part does multiple myeloma usually involve
The skeleton
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Most common primary malignancy of bone
Multiple myeloma
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Age incidence of multiple myeloma
65-75 years
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For the myeloma cells to survive, what do they need?
IL-6
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What are Bence Jones proteins
If only light or heavy chains are produced in a plasma cell dyscrasia, the free light chains are small enough to be excreted in the urine
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What is the mechanism of bone pain in multiple myeloma and what is the result of it
Plasma cells activate RANKL on osteoclasts --> bone destruction --> punched out skeletal lesions on x-Ray
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What is roleux formation and what disease is it seen in
Seen in multiple myeloma: high level of M proteins causes reed cells in peripheral blood smear to stick together in linear formations (can also be seen in lupus)
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Other than the skeletal system, what other organ can multiple myeloma damage
Kidney --> Bence jones proteins can cause myeloma kidney by damaging tubular epithelium
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What areas will multiple myeloma primarily affect
Vertebra (mostly) also rib skull pelvis
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What is seen on bone marrow aspirate for multiple myeloma
Normal cells replaced by plasma cells
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What is found on electrophoresis for multiple myeloma
M spike due to IgG or IgA
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What's the most common plasma cell dyscrasia
Monoclonal gamma patchy of uncertain significance (MGUS)
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MGUS is most likely seen in what age group
Elderly disease. 3% of those older than 50 years and 5% of those older than 70
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What signs are associated with MGUS
M spike on electrophoresis, other features or multiple myeloma are absent. Since it shares a chromosome mutation, it may progress to multiple myeloma so you should monitor it
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What is Waldenström macroglobinemia
B-cell lymphoma with IgM production in a sufficient amount to cause a hyperviscosity syndrome, no Bence jones proteins and no bone destruction
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Symptoms associated with lymphoplasmacytic lymphoma/Waldenström macroglobinemia (4)
Lymphadenopathy, hepatomegaly, splenomegaly, hyperviscosity syndrome
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What is hyperviscosity syndrome
Visual impairment (retinal hemorrhages), neurological problems, bleeding, cryoglobinemia (ie. Raynaud's phenomenon)
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Prognosis of lymphoplasmacytic lymphoma
Incurable progressive disease, median survival of 4 years
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Plasma cell Neoplasm presenting as a solitary lesion of bone or soft tissue
Solitary myeloma/plasma chroma
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Most patients with solitary skeletal plasmacytoma progress to what
Multiple myeloma, if it's extra osseous though, you can prob just resect that
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Mantle cells lymphomas commonly present in what age group and gender
Male 50-60 years old
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Mantle cell lymphomas over express
Cyclin D1
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What genetic abnormality is found in mantle cell lymphoma
t(11,14) translocation of cyclin D1 and the IgH locus
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Most common presenting symptom of mantle cell lymphoma and what is it's prognosis
Painless lymphadenopathy, survival 3-4 years
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What diseases are marginal zone lymphomas associated with?
Inflammatory/autoimmune diseases like Hashimotos, sjögren, and hpylori
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What is the spread and prognosis of marginal cell lymphomas like
Remains localized until really late and the tumor can regress if the inciting agent is taken away
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What does histology look like for hairy cell leukemia
Hairlike projections on peripheral blood smear. They cant really be aspirated (dry tap) because the cells are enmeshed in an extra cellular matrix composed of reticulin fibrils. Otherwise, biopsy is crowed with the neoplastic cells
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What is the clinical finding in hairy cell leukemia
Splenomegaly without lymphadenopathy
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What is the prognosis of hairy cell leukemia and what do you have an increased chance of
Good prognosis with chemotherapy and increased chance of infection
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Cells of hairy cell leukemia are positive for what marker? (Pathoma and rapid review)
Tartrate resistant acid phosphatase (TRAP)
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What infection is associated with adult t-cell leukemia
HTLV-1 human t-cell leukemia retrovirus type 1
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How is HTLV-1 involved in the pathogensis of adult t-cell lymphoma
It activates the TAX protein which activates NFKB, which enhances lymphocyte growth and survival
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Clinical findings in adult t-cell leukemia (5)
Hepatosplenomegaly and lymphadenopathy, skin lesions, hypercalcemia, punched out bone lesions (pathoma/rapid review)
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What kind of cells are related to Mycosis fungoides and Sézary syndrome
CD4+ helper T cells
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What body part does Mycosis fungoides infect?
The T cells infect the skin
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What is the difference between Mycosis fungoides and Sézary syndrome and what is Sézary syndrome's characteristic histology
Sézary syndrome is when skin involvement manifests as a generalized exfoliative erythroderma. There is rarely progression to tumefaction and there is an associated leukemia of Sézary cells (cerebriform nuclei)
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Where does Hodgkin's lymphoma first arise and where does it spread?
In a single node or chain of nodes and then to anatomically contiguous lymphoid tissues
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What are the characteristic cells seen in Hodgkin lymphoma
Reed-Sternberg giant cells
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What are Reed-Sternberg cells
Large b-cells with a bi-lobed nucleus, large inclusion like nucleoli and abundant cytoplasm surrounded by a clear halo
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What are the different types of Hodgkin lymphomas (5)
Nodular sclerosis, mixed cellularity, lymphocyte-rich, lymphocyte depletion, lymphocyte predominance
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Which one of the subtypes of Hodgkin lymphoma is the non classical type
Lymphocyte predominance
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What positive markers does reed-steinberg cells express
CD 15 and CD 30
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What are some variants or Reed-Sternberg cells and what do they look like on histology and in what disease are they seen?
1. Mononuclear variants (single nucleus with a prominent nucleolus)
2. lymphohistocytic variants - L&H (large pale staining multilobed cell - lymphocyte predominant type - popcorn cells)
3. Lacunar cells (multilobed nucleus with many small nucleoli in a clear space with lots of cytoplasm - seen in nodular sclerosing)
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What do you need for the diagnosis of Hodgkin lymphoma
Reed-Sternberg cells in a background of non-neoplastic inflammatory cells
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What is the most common form of Hodgkin lymphoma? And who does it mostly present in
Nodular sclerosis type (70%), females predominance? (Only in pathoma and rapid review, book says equal)
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What lymph nodes does the nodular sclerosis type of Hodgkin lymphoma usually involve
Anterior mediastinal, and cervical and supraclavicular
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What is the histology associated with the nodular sclerosis type of Hodgkin lymphoma
Lacunar variant of Reed-Sternberg and the deposit of collagen in bands that divide the lymph nodes into circumscribed nodules
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Which type of Hodgkin lymphoma has a strong association with EBV and what group of people does it commonly involve
Mixed-cellularity type (70%), older men
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What does histology appear as on the mixed cellular it type of Hodgkin lymphoma
Classic Reed-Sternberg cells and mononuclear variants, eosinophils
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Which type of Hodgkin lymphoma has the best prognosis
Lymphocyte-rich type
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Which is Hodgkin lymphoma is the most aggressive type and what population does it most likely involve
Lymphocyte depletion, elderly and HIV+
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What does the non classical form of Hodgkin lymphoma look like on histology and what markers does it express
LandH variants of Reed-Sternberg cell, typical of germinal center B cells (CD20)
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What population doe the lymphocyte predominant form of hodgkin lymphoma present in
Young male with cervical or axillary nodal enlargement
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What do Reed-Sternberg cells secrete
Cytokines
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Symptoms seen in Hodgkin lymphoma
Fever, chills, night sweats, painless lymphadenopathy
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What age group does acute myeloid leukemia usually present in
15-59, likelihood increases as you go older and peaks at 60
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What do you need to see in the bone marrow to diagnose AML
20% myeloid blasts
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What are auer rods
Seen in AML, they are fused azurophilic granules that are splinter-shaped rod structures present in the myeloblasts, they are peroxide se positive
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What are the classifications of AML
M0-7 French American British classification system
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What is seen in M0 AML and are Auer rods present?
Minimally differentiated, no Auer rods
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M1 AML? Are Auer rods present?
AML without maturation, rare Auer rods
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M2 AML? Auer rods?
AML with maturation, Auer rods present, most common type
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M3 AML? Auer rods?
Numerous Auer rods, possible DIC, t(15;17) translocation, abnormal retinoids acid metabolism, high doses of all trans retinoids acid may induce remission by maturing cells
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M4 AML? Auer rods?
Acute myelomonocytic, Auer rods uncommon
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M5 AML? Auer rods?
Acute monocytic, no Auer rods, gum infiltration, usually lack myeloperoxidase
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M6 AML?
Acute erythroleukemia, Multinucleated erythroblastosis, myeloboasts present
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M7 AML?
Acute megakaryocytic AML, myelofibrosis in bone marrow, increased incidence in of own syndrome in children less than 3 years old
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What is the genetic defect seen in M3 of AML?
t(15;17) translocation of the retinoic acid receptor (RAR) on 17 to 15
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Which form of AML is associated with Down's syndrome
M7 acute megakaryocytic
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What disease has a very high risk of transforming to AML
Myelodysplastic syndrome
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What age group does myelodysplastic disorders typically effect?
Elderly 70 years old
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What is the defect common in all myelodysplastic syndromes
Ineffective hematopoesis and maturation defects
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The bone marrow in myelodysplastic syndromes is most commonly..
Hyper cellular
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In myelodysplastic syndromes a common abnormalities seen in the erythroid cells is _____ and what are they composed of
Ringed sideroblasts, erythroblasts with iron laden mitochondria
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What is seen on CBC for myelodysplastic syndromes
Pancytopenia
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What age group is CML most seen in
50-60 years old
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What is the gene defect seen in CML
t(9;22) translocation on ABL proto oncogene, proto oncogene fuses with the break cluster region (bcr) on chromosome 22 --> Philadelphia chromosome
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Symptoms associated with CML
Splenomegaly, accelerated phase after 3 years (increasing anemia and thrombocytopenia, sometimes with basophil rise) --> 6-12 months = blast crisis (like acute leukemia)
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The same type of mutations seen in CML are seen in what other disease?
ALL
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Polycythemia Vera is a proliferation of _____, and what is the most likely cause of symptoms
Red cells, granulocytes, and platelets. Red cell proliferation causes the symptoms
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What mutation is polycythemia associated with
Tyrosine kinase JAK2
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Symptoms of polycythemia Vera
Due to hyperviscosity of blood: blurry vision, headache, increased venous thrombosis, flushed face, itching, minor hemorrhages
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Treatment for polycythemia Vera
Phlebotomy
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What is the spent phase of polycythemia Vera
Clinical and anatomical features of myelofibrosis develop --> splenomegaly, fibrosis in bone marrow
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Mutation associated with essential thrombocytosis
JAK2 or MPL (receptor tyrosine kinase that's normally activated by thrombopoietin)
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What does peripheral smear reveal for essential thrombocytosis
Abnormally large platelets
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Symptoms associated with essential thrombocytosis
Thrombois, erythromyalgia (throbbing and burning of hands and feet by occlusion of small arterioles by platelet aggregates) --> all similar to polycythemia Vera
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How can you distinguish essential thrombocytosis from polycythemia Vera and marrow fibrosis
The absence of polycythemia and marrow fibrosis
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What is the hallmark of primary myelofibrosis
Development of obliterative marrow fibrosis
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What mutations are present in primary myelofibrosis
JAK2 or MPL
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What causes the marrow fibrosis seen in primary myelofibrosis
Megakaryocytes produce excess PGDF causing marrow fibrosis
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Clinical features of primary myelofbrosis (3)
Splenomegaly (extrameduallary hematopoiesis), leukoerythroblastic smear (tear drop RBC), and immature granulocytes
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What is the characteristic sign seen in langerhans cell histiocytosis
Birbeck granules in the cytoplasm, look like tennis rackets
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What is letterer siwe disease
Malignant proliferation of langerhans cells
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What population would you see letterer-siwe test in? And what clinical signs would be present
Skin rash and cystic skeletal defects in an infant of less than 2 years
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What is the prognosis of letterer-siwe disease
Rapidly fatal because it involves many organs, 5 year survival
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What is eosinophilic granuloma
A benign proliferation of langerhans cells in bone
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What is the common population that eosinophilic granuloma appears in
Adolescent with a pathologic fracture
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What is Hand-Schüller-Christian Disease
Malignant proliferation of Langerhans cells
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What is the classic presentation of Hand-Schüller-Christian disease
Calvrial bone defects, diabetes insipidus, exophthalmos
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What kind of sensation will a patient with an enlarged spleen experience
Dragging sensation in hype left upper quadrant
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What is hypersplenism
Anemia, leukopenia, thrombocytopenia, alone or in combination
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DiGeorges syndrome results in defects of what organs
Thymic hypoplasia and hypoparathyroidism
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A thymoma is a tumor of what type of cells
Thymic epithelial cells
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What location will a thymoma most likely arise in
Anterior mediastinum
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What is seen on histology with an invasive thymoma
They penetrate through the capsule into surrounding structures
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Most common variant of a Thymic carcinoma
Squamous cell carcinoma
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What disease is a thymoma associated with
Myasthenia gravis
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What are symptoms of a thymoma related to?
Impingement on mediastinal structures
