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IHO Mike Rose > Diseases Exam 4 > Flashcards

Diseases Exam 4 Flashcards

1
Q

Chronic Myeloid Leukemia

A

Things you must know:

  • Neutrophilic leukocytosis
  • Basophilia
  • Philadelphia chromosome t(9:22)
  • Three phases

Pathophysiology: t(9:22) creates BCR-ABL fusion protein which is an uncontrolled tyrosine kinase causing defects in normal neutrophil differentiation.

Lab findings: +++ WBC, Neutrophilia w/ left shift, basophilia, low hemoglobin, +platelet count (at first), decreased LAP (leukocyte alkaline phosphatase)

Clinical findings:
Sx: Slow onset, fever, fatigue, night sweats, abdominal fullness
Signs: Splenomegaly, hepatomegaly

Phases: Chronic phasee (stable counts, easily controlled, 3-4 years if untreated) then 50/50 go to Accelerated phase (unstable counts, blast crisis within 6-12 months) or to Blast crisis (acute leukemia, high mortalitiy).

Remission:
Hematologic remission - no splenomegaly, WBC
Cytogenetic - no t(9:22)
Molecular - no BCR/ABL

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2
Q

Chronic Myeloproliferative Disorders

A

Things you must know:

  • Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow
  • Four disorders: CML (neutrophiils), PV (RBCs), ET(platelets), MF (everything)
  • Occur only in adults
  • Long course

Features common to all disorders: Increase WBC w/ left shift, hypercellular marrow, splenomegaly, may evolve into acute leukemia, mutatated tyrosine kinases

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3
Q

Polycythemia vera

A

Things you must know:

  • High RBC (makes blood sludgy)
  • Different from seconary polycythemia (smoker, high-altitude, tumor, etc.) –> look to EPO
  • Thrombosis and hemorrhage
  • Jak-2 mutation

Signs: headache, pruritis, dizziness, thrombosis, infarction

Symptoms: spleno/hepatomegaly, plethora (flushing)

Mutated JAK-2: activity increased in PV. Cells grow on their own. Important for Dx and drug therapy.

Treatments and Prognosis:
Rx: phlebotomy, maybe myelosuppressive drugs
Prognosis: median survival: 9-14 years, death from thrombosis or hemorrhage, leukemic transformation in some patients (not as common as in CML)

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4
Q

Essential Thrombocythemia

A

Things you must know:

  • Very high platelet count in blood (>600,000 –> often over 1,000,000)
  • Can occur in young women
  • Diagnosis of exclusion
  • Thrombosis and hemorrhage

Dx: platelets > 600,000, Hgb
Hg

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5
Q

Chronic Myelofibrosis

A

Things you must know:

  • Panmyelosis –> then marrow fibrosis
  • Extramedullar hematopoeisis
  • Teardrop RBCs

Sx: LUQ fullness, weakness, fatigue, palpitations
Signs: Marked splenomegaly, pallor, tachycardia

Treatment: supportive, maybe myelosuppressive drugs early on
Prognosis: Median survival: 3-5 years, death from marrow failure, leukemic transformation

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6
Q

Multiple Myeloma

A

Things you must know:

  • Monoclonal plasma cell proliferation
  • Monoclonal gammopathy (M-spike)
  • Decreased normal immunoglobulins
  • Osteolytic lesions. (hypercalcemia)

Lab findings: M- spike (generally IgG never IgM), Bence-jones protein (light chains) in urine, decreased normal Ig

Important tests: Serum protein electrophoresis, Serum immunoelectrophoresis, and urine immunoelectrophoresis

Clinical features: bone pain (80%), bruising/bleeding, infx, hypercalcemia (osteolytic lesions), renal failure, hyperviscosity (decreased vision, purpura, confusion.

Classic triad: anemia, bone pain, renal failure.

Average age of Dx: 69 years old.

Major COD: infection or renal failure

Treatment: Dexamethasome, Melphalan, Cyclophosphamide, or autologous peripheral blood stem cell transplant (PBSC), thalidomide, lenalidomide, bortezomib

Support w/ Bisphosphonates, and EPO

Morphology:
Blood: anemia, rouleaux, flame cells, russell bodies, dutcher body, and mott cell.
Marrow: plasma cells, amyloid (congo red stain + polarized lens)

Can develop from MGUS (monoclonal gammopathy of undertermined significance) –> cmall M spike w/o myeloma symptoms.

Prognosis: Conventional chemo 3-4 years. Intensive = variable depending on age

Other plasma cell tumors: Solitary plasmacytoma, plasma cell leukemia, waldenstrom macroglobulinemia, MGUS.

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7
Q

Waldenstrom macroglobulinemia

A
  • Lymphoplasmacytoid lymphoma -lymphoid cells that act plasma cell like
  • Make IgM (give away)
  • Hyperviscosity syndrome–> may present w/ retinal problems.
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8
Q

Chronic lymphocytic leukemia

A

Pathophysiology: CLL is an uncontrolled clonal accumulation of mature lymphocytes. Mutated IgV-H genes. Often BCL-2 gene rearrangement (prevents apoptosis)

Things you must know:

  • Small, mature lymphocytes (=small lymphocytic lymphoma)
  • B cell, but CD5+ (weird!)
  • Long but inexorable course

Dx: Lymphocytosis >5000/ml, flow cytometry (CD5+, CD19+, CD20+, CD23+), cytogenetics (FISH)

KNOW CD5+ and TdT-!

Always get FISH studies w/ CLL.

Sx: progressive adenopathy, fatigue, malaise, weight loss, fevers

CBC = + or +++WBC and hypogammaglobinulinemia

Tough DDX vs. Mantle Cell Lymphoma (Nasty aggressive NHL Key indicator is CD23-)

Autoimmune sequelae and Richter’s transformation are long-term complications (AIHA, Pure red cell aplasia, Idiopathic thrombocytopenic purpura (ITP), neutropenia)

Staging: Rai system. 0-4. Median survival Rai 0 is > 10 years; Rai 1 = 7 years; Rai 3 = 2-5 years

Prognosis: Better if IgV-H mutation, Worse if CD38+, ZAP70+; counts (how are normal cells), adenopathy, BM pattern

Death from infection!

Most common leukemia in adults (older than 40). Median age of dx is 65 years.

Median survival = 9 years.

Rx = Conservative (not always treating) Ibrutinib (very effective, but extreme cost toxicity.)

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9
Q

Rebound Angiogenesis

A

Rapid growth of cancer when an angiogenesis inhibitor is stopped.

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10
Q

Chronic lymphoproliferative disorders

A

Things you must know:

  • Malignant proliferation of lymphocytes in blood, marrow
  • Occur only in adults
  • Many disorders; CLL most important
  • Long course; indolent but incurable
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11
Q

Hairy Cell Leukemia

A

Things you must know:

  • Hairy cells
  • Splenomegaly w/o lymphadenopathy
  • Pancytopenia (and monocytopenia)
  • TRAP stain +

Clinical findings: Older (40-60)
M:F = 5:1
Hypercellular bone marrow w/ moth eaten appearance.
Reticulin fibrosis (chicken-wire appearance)

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12
Q

Prolymphocytic Leukemia

A

Things you must know:

  • Prolymphocytes
  • Splenomegaly w/o lymphadenopathy
  • Rare
  • Aggressive

Labs: ++ WBC, -Hgb, -platelets

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13
Q

Large Granulated Lymphocyte Leukemia

A

Things you must know:

  • Large granulated lymphocytes
  • T-cell
  • Neutropenia
  • Long survival

Clinical findings:

  • Infections (neutropenia)
  • Long survival

Immunophenotype: T cells

Labs: +WBC, Neutropenia

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14
Q

Follicular Hyperplasia

A

Things you must know:

  • Large, irregular follicles
  • Mixture of cells in germinal centers
  • Tingible body macrophages
  • B-cell response to some immune stimulus.
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15
Q

Interfollicular Hyperplasia

A

Things you must know:

  • Expanded area between follicles
  • Mixture of cells
  • Partial effacement
  • T-cell response to some immune stimulus
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16
Q

Non-Hodgkin Lymphoma

A

Things you must know:

  • Malignant proliferation of lymphoid cells (blasts or mature cells) in lymph nodes
  • Skips around the body
  • Many subtypes
  • Most are B cells

Sx: Painless, firm lymphadenopathy; extranodal manifestations,

“B” Sx: weight loss, night sweats, fever

Low grade = older patients, indolent (incurable), small mature cells, non-destructive

High grade = Children sometimes, Aggressive (curable), Big ugly cells, destructive

Classification: Working = Low, Intermediate, High grade; REAL/WHO = B-cell vs. T-cell.

Types:
Low Grade = SLL, Malt lymphoma, Follicular lymphoma, mycosis fungoides
High Grade = Large cell lymphoma, Lymphoblastic lymphoma, Burkitt lymphoma

17
Q

Small lymphocytic lymphoma

A

Things you must know:

  • Small mature lymphocytes
  • Same disease as CLL
  • B-cell lesions, but CD5+
  • Long course, death from infx

Richter’s transformation = bad

18
Q

Marginal Zone Lymphoma

A

Things you must know:

  • Actually a bunch of lymphomas
  • Marginal zone pattern
  • Malt lymphoma
  • H. pylori
19
Q

Mantle Cell Lymphoma

A

Things you must know:

  • Mantle zone pattern
  • Small angulated lymphocytes
  • t(11:14) - bcl-1 and IgH; Cyclin D1 positive
  • Aggressive and Nasty

Ddx vs CLL = Mantle cell = Cd23-

20
Q

Follicular lymphoma

A

Things you must know:

  • Follicular pattern
  • Small cleaved cell, mixed or large cell
  • Grade 1, 2, or 3
  • t(14:18) and - IgH and bcl-2

Butt cells!

Stage I = Single node (90% 5 yrs.)
Stage II = Two or more nodes on same side of diaphragm (90% 5 yrs.)
Stage III = Lymph nodes on both sides of (40% 5 yrs.)
Stage IV = Diffuse extranodal involvement (40% 5 yrs.)

A= no additional symptoms
B = weight loss, night sweats, fever
21
Q

Mycosis Fungoides/Sezary Syndrome

A

Things you must know:

  • Skin lesions
  • Blood involvement
  • Cerebriform lymphocytes
  • T-cell immunophenotype.

Histo: Pautrier microabscess in skin; Sezary cells (cerebriform appearance)

22
Q

Diffuse Large Cell Lymphoma

A

Things you must know:

  • Large B cells
  • Extranodal involvemnet
  • Grows rapidly –> poor prognosis
23
Q

Lymphoblastic Lymphoma

A

Things you must know:

  • Two types: B and T
  • Lymphoblasts in diffuse pattern
  • Same as ALL
  • T-lymphoblastic lymphoma often in teenage male w/ mediastinal mass.
24
Q

Burkitt Lymphoma

A

Things you must know:

  • Child w/ fast-growing, extranodal mass
  • Starry-sky pattern (has tingible body macrophages)
  • t(8:14) (c-myc)
  • Same as Burkitt leukemia

African type (jaw) and non african type (abdominal mass)

25
Q

Adult T-cell Leukemia/Lymphoma

A

Things you must know:

  • Japan/Caribbean basin (moving into US. Florida now?)
  • HTLV-1
  • Skin lesions, hypercalcemia
  • very aggressive
26
Q

Hodgkin Lymphoma

A

Things you must know:

  • Younger patients, good prognosis
  • Contiguous spread (not jumping)
  • Five subtypes
  • Reed Sternberg cell (One huge giant cell. Giant nuclei with distinct large nucleoli (Owl’s eye))
Subtypes: 
Nodular lymphocyte predominance Hodgkin lymphoma (best outcome ---> young healthy male)
Classical Hodgkin lymphoma:
-Nodular sclerosis
-Lymphocyte rich
-Mixed cellularity
-Lymphocyte depletion

Prognosis is based on stage, not subtype. Subtypes have different prognosis based on when they are normally caught not on the virulence of the disease.

Rx and Prognosis:

  • Surgery, chemo, radiation
  • Prognosis depends on stage
  • Concern about creating secondary leukemia
27
Q

Nodular L-P Hodgkin Lymphoma

A

Things you must know:

  • Asymptomatic young male with cervical lymphadenopathy
  • Good prognosis
  • B cell origin
  • Popcorn cells
28
Q

Nodular Sclerosis Hodgkin Lymphoma

A

Things you must know:
Most common subtype
Good prognosis (early stage)
Lacunar cells

29
Q

Mixed cellularity Hodgkin Lymphoma

A

Things you must know:

  • Worse prognosis
  • Usually disseminated at presentation
  • Classic Reed-Sternberg cells
  • Mixture of background cells.
30
Q

Lymphocyte-Rich Hodgkin Lymphoma

A

Things you must know:

  • Uncommon
  • Usually localized at presentation
  • Popcorn cells
31
Q

Lymphocyte Depletion Hodgkin Lymphoma

A

Things you must know:

  • Rare
  • Often disseminated at presentation
  • Classic Reed-Sternberg cells
  • Collagen or reticulin background.
32
Q

Bruton’s Agammaglbulinemia or XLA immunodeficiency

A

Mutated BTK gene encoding the enzyme Bruton’s Tyrosine Kinase.

Key enzyme involved in signal transduction downstream of the pre-BCD and BCr.

Mutated in immunodeficiency XLA. –> very few circulating B cells and negligible serum Ig.

85% of agammaglobulinemia (AKA Bruton’s Agammaglobulinemia)

33
Q

MHC I deficiency

A

Causes decreased amount of CD8+ T cells.

Increased susceptibility to viral infections.

Can be due to a deficiency in TAP proteins. (autosomal recessive)

34
Q

MHC II deficiency

A

A mild form of SCID that is due to a failure of gene regulation.

Onset after 6 months.

Hypoglobulinemia.

Low CD4+ counts.

Autosomal recessive trait.

Treat w/ Hematopoetic stem cell trasnplant (HSCT).

35
Q

Common variable immunodeficiency CVID

A

Impacts later stages of B-cell development.

Results in reduced serum Ig, memory B cells, class switch recombination, and B-cell activation

Mutations in CD40 ligands on T-cells, CD-19, and others have been implicated in CVID patients.

IHO Mike Rose (20 decks)

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