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144 Cards in this Set
- Front
- Back
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What is this disease?
NADPH deficiency |
CGD (chronic granulomatous disease): autosomal recessive.
* X-linked: cytochrome b deficiency. |
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How to diagnose chronic granulomatous disease (CGD)?
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NBT dye reduction
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What can be used to treat congenital neutropenia?
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G-CSF
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What are some conditions/processes that cause increased destruction of neutrophils?
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- complement-induced granulocyte aggregation
- hypersplenism - immune neutropenia |
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Which substance can shift granulocyte (neutrophils) from the margination to the circulating pool?
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epinephrine
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Which substance inhibit granulocyte migration to the tissue?
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steroids
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Give a main difference between the content of azurophilic granules and specific granules.
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Azurophillic granule: peroxidase +, MPO, defensins.
specific granule: peroxidase - |
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The tertiary granule in neutrophils contain ____.
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gelatinase
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Which interleukin activates neutrophils?
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IL8
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What are some types of chemical mediators of inflammation?
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- vasoactive amines: histamine, serotonin
- plasma proteases: complement, kinin, clotting system - PLA2 metabolite: prostaglandins, leukotrienes. - cytokines: IL1, TNF, IL8 - lytic enzymes/oxygen radicals |
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List 3 causes of neoplastic leukocytosis.
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- leukemia
- lymphoma - myeloproliferative disorders |
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What disease is this?
- overproduction of progenitor cells in the bone marrow. - clear block in differentiation of stem/progenitor cells. - clonal disorder |
acute leukemia
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What disease is this?
- overproduction of progenitor cells in the bone marrow. - no clear block in maturation of stem/progenitor cells. - heterogenous collection of immature cells and mature cells(functional). |
chronic leukemia
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Name a disease that could be classified as both chronic leukemia and myeloproliferative disorder.
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chronic myelogenous leukemia: proliferation of both immature and mature cells.
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What disease is this?
- cancer of lymphoid organ - overproduction of lymphoid cells in lymph nodes, spleen, other lymphoid tissues. - cell type and aggressiveness are more important to therapy. |
lymphoma
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2 types of chromosomal abnormalities in leukemia.
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- qualitative: translocations, inversions, insertions.
- quantitative: change in chromosome number (hyper- or hypo- diploidy) |
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What disease is this?
- overproduction of one or more mature, myeloid cells. - clonal disorder |
myeloproliferative disorder
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Give 2 acute leukemia.
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- acute lymphoblastic leukemia (ALL)
- acute myeloid leukemia (AML) |
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What disease is this?
- overproduction of one or more mature, myeloid cells. - non-clonal disorder |
reactive leukocytosis
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Why is acute leukemia fatal if left untreated?
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Overtime, leukemic blasts diffusely replace bone marrow and infiltrates liver, spleen, lymph nodes. Suppression of stem cells will produce pancytopenia which causes fever, infection and hemorrhages.
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Give 2 chronic leukemia.
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- chronic mylogenous leukemia (CML)
- chronic lymphocytic leukemia (CLL) |
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Give 2 lymphomas.
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- Hodgkin's disease
- non-Hodgkin's lymphoma |
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What 3 techniques can be used to detect lymphoid neoplasms?
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- morphology (microscopic)
- immunopathology (surface antigens) - molecular genetics (translocations) |
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What is this disease?
- marrow hyperplasia, hypermetabolism due to increased cell turnover - cytogenetic abnormalities - abnormal maturation |
myeloproliferative disorder
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Name a myeloproliferative disorder of erythrocyte.
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polycythemia vera
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Name a myeloproliferative disorder of granulocyte.
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chronic myelogenous leukemia
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Name a myeloproliferative disorder of platelets.
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essential thrombocythemia (ET)
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Name a myeloproliferative disorder of fibroblast.
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agnogenic meyloid metaplasia (AMM)/ idiopathic myelofibrosis
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What is this disease?
What is the abnormal protein? t(9, 22) |
CML or Adult B-ALL
bcr(22)/abl(9) |
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What is this disease?
What is the abnormal protein? t(8, 21) |
AML
CBF/AML1 |
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What is this disease?
What is the abnormal protein? t(15, 17) |
APL
RAR-alpha/PML |
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What is this disease?
What is the abnormal protein? t(8, 14) |
Burkitt's lymphoma
Ig/myc |
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Which chromosome translocation will produce abnormal protein bcr/abl in CML?
How does the abnormal protein cause this disease? |
t(9, 22)
increased tyrosine kinase activity that results in increased transcription. |
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Which chromosome translocation will produce abnormal protein CBF/AML1 in AML? How does the abnormal protein cause this disease?
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t(8, 21)
CBF/AML1 forms a complex with corepressor so that the downstream genes for differentiation are blocked. |
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Which chromosome translocation will produce abnormal protein RAR-alpha/PML in APL?
How does the abnormal protein cause this disease? |
t(15, 17)
RAR-alpha/PML is a mutated form of RA receptor that no longer able to bind with RA(vitamin A) and proceed the transcription process of the differentiation. |
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Which chromosome translocation will produce abnormal protein Ig/myc in Burkitt's lymphoma?
How does the abnormal protein cause this disease? |
MYC(8) tranlocations:
- t(8, 14) Ig/myc leads to myc overexpression that results in B cell proliferation. - t(2,8) - t(8, 22) |
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What is the activity of the abnormal protein bcr/abl in CML? What is the new discovery for its treatment?
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increased tyrosine kinase activity
STI-571: tyrosine kinase inhibitor |
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How to treat APL?
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ATRA (all-trans retinoic acid): overturn the effects of mutated RAR from t(15, 17) by inducing terminal differentiation.
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Presence of what can be used to differentiate ALL from APL?
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Presence of Auer rods: think APL (acute promyeloctic leukemia). Rule out lymphoid lineage.
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MPO exists in ___.
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hypergranular promyelocyte.
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Leukocyte alkaline phosphatase is high in ____.
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high in leukemoid reaction
low in leukemia |
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The following are associated with what cell lineage?
- CD3 - CD4 - CD8 |
T cell
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The following are associated with what cell lineage?
- CD10 - CD19 - CD20 |
B cell
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The following are associated with what cell lineage?
- CD11c |
monocyte and granulocyte
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The following are associated with what cell lineage?
- CD34 |
pluripotent stem cell
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The following are associated with what cell lineage?
- CD14 |
monocyte
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The following are associated with what cell lineage?
- CD15 |
granulocyte
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The following are associated with what cell lineage?
- CD22 |
resting B cell
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Which antigen is expressed on both developing and mature B cell but not on plasma cells?
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CD19
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Which antigen is expressed on developing B cell and is also called CALLA?
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CD10
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Antibodies anti-CD33 can be used to treat ____.
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AML
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Rituxan, anti-CD20(chimeric), can be used to treat ____.
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NHL
anti-CD20 binds to CD20 on B cells and induce ADCC and complement dependent cytotoxicity. |
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What is this disease?
- Flow cytometry: +TdT, HLA-DR, CD19, CD10, cytoplasmic 79a - cytogenetics t(9,22), t(12,21), t(4,11), t(1,19) |
B-cell ALL
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What is this disease?
- Flow cytometry: +TdT, CD7, CD3 - cytogenetics translocation of ch7, ch14 with MYC(8), HOX11(10), or LCk(1) |
T-cell ALL
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What is this disease?
- Flow cytometry: +CD13, CD33, CD19, CD56, CD34 |
AML with t(8,21)
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What is this disease?
- Flow cytometry: +CD13, CD33 - HLA-DR, CD34 |
APL t(15,17)
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What is this disease?
- Flow cytometry: +CD7, CD56 |
therapy-related AML
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What diseases will show +CD5 on flow cytometry? How to differentiate them apart?
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- CLL: also CD23+, but cyclin D1 negative
- Mantel cell lymphoma: CD23-, +cyclin D1. - T-cell neoplasms (eg. mycosis fungoides): will also have CD3+, CD4+. |
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What diseases will show "starry-sky" appearance morphologically?
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- ALL
- Burkitt Lymphoma |
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What diseases are associated with bone pain?
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- B-cell ALL
- Multiple Myeloma |
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When FISH result shows t(9,22), what diseases would you suspect?
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- B-cell ALL
- CML |
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Which disease shows "smudged cells"?
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CLL
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What important transcription factor is located on chromosome 8?
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MYC
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What important gene is located on chromosome 11?
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cyclin D1
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What important gene is located on chromosome 14?
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IgH
TCR: alpha, delta (involved with T-ALL) |
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What important gene is located on chromosome 7?
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TCR: beta, gamma (involved with T-ALL)
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What important gene is located on chromosome 10?
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HOX11 (involved with T-ALL)
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What is this disease?
- flow cytometry: +CD19, CD20, CD5, CD23 - cytogenetics: trisomy 12 |
CLL
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What is this disease?
- flow cytometry: +CD19, CD20, CD5, CD23 - cytogenetics: 11q del |
CLL
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What is this disease?
- flow cytometry: +CD19, CD20, CD5, CD23 - cytogenetics: 17q del |
CLL
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What is this disease?
- flow cytometry: +CD19, CD20, CD5, CD23 - cytogenetics: 13q14 del |
CLL
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In CLL patients, prognosis is usually good except having these genetic defects.
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11q and 17q deletion.
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What is this disease?
- flow cytometry: +CD5, CD19, CD20, cyclin D1 - CD23 - cytogenetics: t(11,14) |
Mantle cell lymphoma
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What is this disease?
morphology: - small lymphocytes with angulated(cleaved) nuclei - atrophied germinal center |
Mantle cell lymphoma
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What is this disease?
morphology: - Russel and Dutcher inclusions |
Lymphoplasmacytic lymphoma: Igs in the inclusions.
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What is this disease?
Genetics: t(9,14) del 6q |
Lymphoplasmacytic lymphoma
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What is this disease?
Genetics: PAX rearrangement |
Lymphoplasmacytic lymphoma
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How to manage Lymphoplasmacytic lymphoma?
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plasmaphoresis
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What is the cause of itchy skin, diarrhea, bleeding, and visual impairment seen in Lymphoplasmacytic lymphoma?
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itchy skin: Ig deposit in the skin
diarrhea: Ig deposit in GI tract bleeding: interaction of Ig with clotting factors visual impairment: retinal vein distention/hemorrhage due to hyperviscosity. |
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What do you find in the serum of patients with lymphoplasmacytic lymphoma?
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eleveated IgM, >30 gm/dl
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What is this disease?
Genetics: trisomy 13 flow cytometry: +CD19, CD20, CD79a |
MALT
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What is this disease?
Genetics: t(1,14) t(11,18) flow cytometry: +CD19, CD20, CD79a |
MALT
*t(11,18) resistant to H. pylori eradication therapy. |
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What is this disease?
small monoclonal Ig (IgM) flow cytometry: +CD19, CD20 |
Splenic Marginal zone lymphoma
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What treatments are available for splenic marginal zone lymphoma?
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- splenectomy
- rituximab(anti-CD20) - chemotherapy |
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What is this disease?
flow cytometry: +CD11c, CD19, CD20, CD22, CD23, CD103 |
Hairy cell leukemia
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What's the treatment for hairy cell leukemia?
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purine necleoside (2-chlorodeoxyadenosine)
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Which leukemia involves the white pulp of the spleen, which involves the red pulp of the spleen?
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white pulp: splenic marginal zone lymphoma
red pulp: hairy cell lymphoma |
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What is the most common non-Hodgkin's lymphoma in the US?
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Follicular lymphoma
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What is this disease?
-flow cytometry: +CD19,CD20,CD10, BCL-2 |
Follicular lymphoma
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What is this disease?
-cytogenetics: t(14,18) |
Follicular lymphoma
30% Diffuse Large B-cell Lymphoma |
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What viruses may cause immunedeficiency-associated large B-cell lymphoma?
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- HIV
- EBV |
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What viruses may cause body cavity large B-cell lymphoma?
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- HIV
- HHV8 |
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What is this disease?
- flow cytometry: +CD19, CD20, PAX-5, CD10 |
Diffuse Large B-cell Lymphoma
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What is this disease?
- genetics: abnormal BCL-6 expression (3q26) |
Diffuse Large B-cell Lymphoma
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What is this disease?
- morphology diffuse sheets of large lymphocytes in rapidly enlarging lymph nodes |
Diffuse Large B-cell Lymphoma
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What is this disease?
- flow cytometry: +CD19, CD20, CD22, CD20, BCL-6 - cytogenetics t(8,14) |
Burkitt lymphoma
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What is this disease?
- flow cytometry: +CD19, CD20, CD22, CD10, BCL-6 - cytogenetics t(2,8) |
Burkitt lymphoma
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What is this disease?
- flow cytometry: +CD19, CD20, CD22, CD20, BCL-6 - cytogenetics t(8,22) |
Burkitt lymphoma
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What are the 3 types of Burkitt Lymphoma?
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- endemic: lumpy jaw
- sporadic: abdominal - immunodeficiency associated |
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What is this disease?
- morphology: starry sky appearance median sized lymphocytes with cytoplasmic vacuoles. |
Burkitt lymphoma
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What is this disease?
low serum Ig high urine monoclonal Ig (Bence-Jones proteins) |
multiple myeloma
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What is this disease?
morphology: plasma cell: multiple nuclei, cytoplasmic droplets of Igs |
multiple myeloma
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What is the disease?
2.2 serum monoclonal protein no Bence Jones protein |
MGUS
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What is the disease?
monoclonal free light chains, mainly lambda. |
Primary amyloidosis
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Name some lymphomas associated with HIV.
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- Burkitt lympnoma
- Diffusse large B-cell lymphoma - primary effusion lymphoma: HHV8 - plasmablastic lymphoma: EBV - Hodgkin lymphoma |
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What is this disease?
- small-median size prolymphocytes - mature post-thymic T cell in periphery - aggressive disease: <1 yr |
T-cell prolymphocytic leukemia
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What can be used to treat T-cell prolymphocytic leukemia?
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Anti-CD52 (CAMPATH-1H)
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What is this disease?
- peripheral large granular lymphocytes - neutropenia, anemia - no hepatomegaly - no lymphadenopathy |
T-cell Large Granular Lymphocytic Leukemia
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What is this disease? and what is a possible underlying cause?
- rheumatoid arthritis - neutropenia - spleenomegaly |
This is Felty syndrome, may have T-cell large granular lymphocytic leukemia as an underlying cause.
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What is the clinical course of T-cell large granular lymphocytic leukemia?
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indolent, only treat with corticosteroids if symptomatic.
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What is this disease?
HTLV1 infection "flower" cells skin lesion hypercalcemia |
Aldult T-cell leukemia
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What does HTLV1 infection cause?
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- Aldult T-cell leukemia
- demyelinating desease of the CNS and spinal cord |
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What are the stages of mycosis fungoides?
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- premycotic phase
- plaque phase - tumor phase |
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What is this disease?
- morphology: Pautrier's miroabscess cerebriform nuclei |
mycosis fungoides
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What is this disease?
- flow cytometry: +CD2, CD3, CD4, CD5 -CD7, CD8 |
mycosis fungoides
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Which of the following disease is limited to the skin?
- Adult T-cell leukemia - Mycosis fungoides - Sezary syndrome |
- Mycosis fungoides
- Sezary syndrome |
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What is this disease?
- generalized exfoliative erythroderma - rarely proceed to tumefaction |
Sezary syndrome
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Hodgkin vs. non-Hodgkin lymphoma:
orderly spread |
Hodgkin lymphoma
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Hodgkin vs. non-Hodgkin lymphoma:
extranodal involvement uncommon |
Hodgkin lymphoma
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Hodgkin vs. non-Hodgkin lymphoma:
Reed-Sternburg cell seen |
Hodgkin lymphoma
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Hodgkin vs. non-Hodgkin lymphoma:
more oftern localized to a single axial group |
Hodgkin lymphoma
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Hodgkin vs. non-Hodgkin lymphoma:
Waldeyer's ring and mesenteric lymph nodes rarely involved |
Hodgkin lymphoma
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What does Reed-sternberg cell do in Hodgkin lymphoma?
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secrete cytokins (IL5, IL6, IL13, TNF, GM-CSF) to induce reactive infiltration of lymphocytes, granulocytes and monocytes.
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Name the 4 classical subtypes of Hodgkin lymphoma and their distinctive morphological features.
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1. Nodular sclerosis type: lacunar cell, collagen bands
2. Mixed cellularity type: Reed-sternberg cell, mononuclear variant 3. Lymphocyte-rich type: reactive lymphocytes, Reed-Sterberg cells, monocytes. 4. Lymphocyte-depeleted type: Reed-sterberg cells, little lymphocytes. |
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What is the major difference between the classical and nonclassical Hodgkin lymphoma?
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Classical type:
+CD15, CD30 -CD20, -CD45 Nonclssical type: +CD15, CD30, CD20, BCL-6 -CD45 |
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Which of the following type of Hodgkin lymphoma is not related to EBV?
1. Nodular sclerosis type 2. Mixed cellularity type 3. Lymphocyte-rich type 4. Lymphocyte-depeleted type 5. Lymphocyte predominant type |
1. Nodular sclerosis type
5. Lymphocyte predominant type |
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Which of the following type of Hodgkin lymphoma has worse prognosis?
1. Nodular sclerosis type 2. Mixed cellularity type 3. Lymphocyte-rich type 4. Lymphocyte-depeleted type 5. Lymphocyte predominant type |
4. Lymphocyte-depeleted type
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What is this disease?
Cytogenetics: t(9,22) CBC: neutrophilia, eosinophilia, basophila |
CML
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What is the treatment for CML?
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Gleevec: tyrosine kinase inhibitor, but does not cure the disease. If there is <3 log reduction in fusion protein, then consider bone marrow transplant.
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What is this disease?
Hg=17(f) JAK2 mutation |
polycythemia vera
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What is this disease?
Hg =19(m) JAK2 mutation |
polycythemia vera
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What is this disease?
- clonal myeloproliferation of megakaryocytes - JAK2-V617F mutation - sustained thrombocytosis |
Essential Thrombocytosis
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What is this disease?
- megakaryocyte and granulocyte proliferation in bone marrow - tear drop red blood cell - reticulin fibrosis in bone marrow. |
Chronic idiopathic myelofibrosis
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What is this disease?
Risks for: - bone marrow failure - thromboembolic event - portal hypertension - cardiac failure - transform to AML |
Chronic idiopathic myelofibrosis
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What is this disease?
- megakaryocyte proliferation and atypia - reticulin fibrosis - JAK2 mutation |
Primary myelofibrosis
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What is a ringed-sideroblast?
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erythroblast with iron-laden mitochondria (MDS)
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What is a bi-lobar neutrophil called?
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pseudo-pelger huet cell (MDS)
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What is a pseudo-pelger huet cell?
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bi-lobar neutrophil (MDS)
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What is this disease?
-morphology: multiloblar nucleus in red progenitor cells megakaryocytes with multiple nuclei |
MDS
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Which genetic defect confer good prognosis in patients with MDS?
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5q deletion
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What important gene is located on chromosome 18?
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BCL-2
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PAX-5 gene is involved in which 2 disease?
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- lymphoplasmacytic lymphoma
- diffuse large B-cell lymphoma |
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What important gene is located on chromosome 3q26?
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BCL-6 (diffuse large B-cell lymphoma)
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What gene is located on chromosome 9?
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PAX-5
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