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Pathology Post Midterm > Module 1: Heme: Plasma Cell Neoplasms + ALL + AML > Flashcards

Module 1: Heme: Plasma Cell Neoplasms + ALL + AML Flashcards

1
Q

Now to start with Plasma Cell Neoplasms: The first is Monoclonal Gammopathy of Undetermined Significance (MGUS): term applied to an asymptomatic monoclonal gammopathy. What are some features?

A 
M proteins (IgG or IgA) are found in the serum of 1% to 3% of otherwise healthy persons older than age 50 years. 
--precursor lesion with a tendency to evolve to multiple myeloma
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2
Q

Describe the M spike see in MGUS.

A

M spike (IgG or IgA) is smaller than multiple myeloma b/c less plasma cells and Igs)

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3
Q

What symptoms are seen in patients with MGUS?

A

Asymptomatic but there is a small chance of transformation to multiple myeloma
(less than 10% blasts)

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4
Q

The next plasma cell neoplasm is Waldenstrom. What are some features?

A

M spike: IgM (heaviest Ig) —- leads to hyperviscosity syndrome (slows bloodflow — thrombosis —–infarction —stroke, Mi)
Platelets are used up so you get bleeding

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5
Q

What is the treatment for Waldenstrom?

A

Tx: plasmapheresis which removes IgM from serum

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6
Q

Next plasma cell neoplasm is Multiple Myeloma. What is the etiology for this?

A

Plasma cell neoplasm: malignant proliferation of B cells that retain the ability to differentiate into plasma cells and secrete antibodies and suppress the humoral immunity

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7
Q

What are the translocations and pathogenesis for multiple myeloma?

A

Malignant proliferation of a single clone of plasma cells in the bone marrow

  • -t11;14: overexpression of Cyclin D1 and D3
  • -t4;14: 15% of patients and involves fibroblast growth receptor 3 (FGFR3)
  • -produces large amounts of IgG (55%) and IgA (25%)
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8
Q

What are the favored bones in Multiple Myeloma ?

A

Lumbar spine, Ribs and Skull

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9
Q

What demographic of ppl do you see with multiple myeloma?

A

Older males and in people of African Origin

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10
Q

What is the presentation for multiple myeloma?

A
  1. Punched out lytic lesions: due to plasma cells secreting IL-6 showing osteoclast activating factor — results in hypercalcemia – most common symptom is back pain –pathological fractures
  2. Generalized swelling: Anasarca (Generalized edema) due to AL amyloidosis in the kidney
  3. Fatigue: normochromic normocytic anemia (plasma cells take over bone marrow)
  4. Neutropenia: bone marrow invasion
  5. Bleeding: from thrombocytopenia (bone marrow invasion)
  6. Hepatosplenomegaly: due to extramedullary hematopoiesis (liver and spleen trying to make more hematopoietic cells)
  7. Restrictive cardiomyopathy: AL amyloid deposition
  8. Recurrent Pyelonephritis
  9. Renal Dysfunction: due to obstruction via Bence Jones Proteins
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11
Q

What do you see on bone marrow biopsy for patients with multiple myeloma?

A

Most accurate test:
greater than 10-20% plasma cells
Aspirate:
–increased plasma cells with prominent nucleoli, perinuclear halo of clear cytoplasm.

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12
Q

What do you see on serum protein electrophoresis and skeletal xrays on patients with multiple myeloma?

A

Serum Protein Electrophoresis: M spike most frequent M protein produced by myeloma cells is IgG, followed by IgA
Skeletal Xrays: xray all bones in the body look for punched out lesions

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13
Q

What do you see on blood work and urine for a patient with multiple myeloma?

A

Lab:
Hypercalcemia, elevated BUN/Creatinine, Elevated Uric Acid due to turnover of plasma cells produces uric acid — gout
Elevated ESR
Urine:
Bence Jones Proteins: Malignant plasma cells secrete both complete immunoglobins and free light chains and thus produce M proteins and Bence Jones Proteins

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14
Q

What are the complications of multiple myeloma?

A

Complications:

  • -most common cause of death is recurrent infections
  • -2nd most common is renal failure
  • -Amyloid deposition: restrictive cardiomyopathy, carpal tunnel, and macroglossia
  • –CHF due to anemia
  • –Hyperviscosity syndrome: too many immnuoglobins in the blood leads to aggregation of platelets – thrombosis —bleeding
  • -Spinal Cord compression –paralysis
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15
Q

What does CRAB stand for in multiple myeloma?

A 
CRAB: 
HyperCalcemia 
Renal Insufficiency 
Anemia 
Bone lytic lesions/Back Pain
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16
Q

The next topic we are going to discuss is Acute Lymphoblastic Leukemia. What type of people do we see this in?

A

Children: Especially Down’s Syndrome over the age of 5

AML is Downs syndrome patients under the age of 5

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17
Q

What are the two different types of Acute Lymphoblastic Leukemia (ALL)?

A

B cell type (85%): childhood
—Tests, CNS (headaches, blurred vision and vomiting)
T cells (15%)- adolescent males
–NOTCH-1 expression
–worse prognosis
—mediastinal lymphadenopathy on xray (enlarged thymus)
–CD2 to CD8

18
Q

What is the presentation for a patient with ALL?

A
  1. Pancytopenia: of course recurrent infections and thrombocytopenia (stuck in lymphoblasts so they are taking over the bone marrow and causing pancytopenia)
  2. Hypogammaglobulinemia (Stuck as blasts so expansion)
  3. Bone pain/tenderness (due to the lymphoblasts take over)
  4. Hepatosplenomegaly + Lymphadenopathy
    due to extramedullary hematopoiesis
  5. Lymphocytopenia
19
Q

What do you see on peripheral blood smear in a patient with ALL?

A

PAS (lymphoblasts contain glycogen) and TdT positive lymphoblasts (marker for pre-T and pre-B cells)

20
Q

What do you see on flow cytometry in a patient will ALL?

A

B cell: CD10,19,20
T cell: CD2-8
–this is how you differentiate the two

21
Q

What do you see on bone marrow aspirate of patient with ALL?

A

Bone marrow aspirate:

greater than 20-25% lymphoblasts

22
Q

What are the complications for a patient with ALL?

A

Recurrent infections: most common cause of death (due to the neutropenia and hypogammaglobinemia because lymphoblasts dont make immunoglobins)
B cell: CNS infiltration (meningitis, encephalitis) , testes infiltration (balls and brains) –this is not good because treatment doesnt typically cross the barriers
Give methotrexate treatment , response is good

23
Q

What are indicators a good prognosis for a patient with ALL?

A 
Age: 2-10 years old 
WBC count: high because the lymphoblasts count as WBC even though there is high WBC there is still pancytopenia (due to the neutropenia) 
Immunophenotype: B cell 
Cytogentics: Hyperploidy t(12;21) 
Gender: Girls
24
Q

The next Leukemia to be discussed is Acute Myeloblastic Leukemia (AML). What population is affected?

A

More common in older adults (over 50)

25
Q

There are 8 different types of AML, however, two types are important. AML- Acute promyelocytic leukemia (M3) is the first type to be discussed, what is the pathogenesis for this?

A

AML M3: t(15,17); deletions have worse prognosis

  • -translocation of the RAR
  • -fusion of Vit A receptor
26
Q

How do patients with M3 present?

A
  1. Pancytopenia from bone marrow invasion of promyelocytes
  2. Heptaosplenomegaly due to organ infiltration by the promyelocytes and lymphadenopathy
  3. Recurrent Infections
  4. Thrombocytopenia
27
Q

What tests can be done for AML M3?

A

Test:
FISH most accurate diagnostic test
Bone marrow aspirate: greater than 20-25% blasts (immature myeloblasts)
Bone marrow biopsy hypercellular
Peripheral Blood Smear: lots of myeloblasts with Auer Rods –needle shaped in the cytoplasm , used MPO stain and cytoplasm appears black .

28
Q

What are the complications for AML M3?

A
  1. Recurrent Infections most common cause of death
  2. Heart Failure: Anemia
  3. Do not give chem
  4. Auer rods are thrombogenic — DIC
  5. Waterhouse Friderichsen Syndrome: DIC + septic shock + bilateral adrenal hemorrhages
  6. Destruction of renal cortex can lead to Addison’s Disease
  7. Schistocytes on blood smear: also see them with HUS and TPP
29
Q

What is the treatment for AML M3?

A

ATRA (All trans retinoic acid)

–patients tend to have a favorable response to this

30
Q

The next type of AML is – Acute Monocytic Leukemia (M5), what is the main symptom associated with this type of AML?

A

Usually detected first by dentists and dermatologists

–Gingival hyperplasia

31
Q

Finally for any AML what do you see on blood work?

A 
Increased bleeding time 
Increased PT and PTT
Increased D-dimers 
Decreased platelets 
Decreased fibrinogen
32
Q

What diseases can transform to AML?

A 
Myeloproliferative disorders (blast crisis) 
--CML (most common) 
-MDS
--Essential thrombocytosis
--Polycythemia vera 
--Myelofibrosis 
CLL 
PNH- can also transform to MDS
33
Q

Moving on to Myelodysplastic syndromes, what are some common features?

A

The bone marrow is partly or wholly replaced by the clonal progeny of a transformed multipotent stem cell that retains the capacity to differentiate into red cells, granulocytes, and platelets , but in a manner that is ineffective and disordered.

  • -marrow is hypercellular or normocellular
  • -Peripheral blood smear: one or more cytopenias
34
Q

What are the two types of Myelodysplastic Syndromes (MDS)?

A
  1. Idiopathic/primary MDS: greater than 50, gradual onset
  2. Therapy-related MDS with suppression of myeloid precursor cells — neutropenia: 2-8 years post chemotherapy (ex: low stage Hodgkin’s lymphoma o radiotherapy for 8 years; dysgermination, seminoma or medullary carcinoma of the breast)
    - -can also get papillary carcinoma of the thyroid post ionizing radiation
35
Q

What is the outcome of myelodysplastic syndrome?

A

Transformation to AML or death related to complications of cytopenias

36
Q

What are the karyotypic abnormalities for MDS?

A

Monosomy 5 or 7; deletions of 5q,7q,20q

37
Q

What is the treatment for MDS?

A

Allo-BMT in younger patients

Supportive therapy in older patients

38
Q

Next we are going to focus on the chronic myeloproliferative neoplasms. What is the common pathogenic feature?

A

Mutated, constitutively activated TKs circumvent normal growth controls

  • –proliferation of mature cells of myeloid lineage (high WBC with hypercellular bone marrow)
  • -seen in late adulthood (over 50)
39
Q

What are the four major entities (neoplasms) associated with chronic myeloproliferative neoplasms?

A
  1. Chronic Myeloid Leukemia: mutation in t(9;22) Philadelphia, BCR-ABL, fusion gene; with a 100% frequency, result = constitutive ABL kinase activation
  2. Polycythemia Vera: JAK2 point mutation with over 95% frequency, result = constitutive JAK2 kinase activation
  3. Essential Thrombocythemia: JAK2 point mutations, MPL point mutations with 50-60% frequency. Result = constitutive kinase activations
  4. Primary Myelofibrosis: JAK2 point mutations, MPL point mutations with 50-60% frequency. Result= constitutive kinase activation
40
Q

What other condition in patients will give them lytic lesions?

A

Adult T cell leukemia

  • –caused by HTLV very common in Japan and Caribbean.
  • -Lytic lesions and hypercalcemia
  • -difference is that these ppl get a rash because it infiltrates their skin

Pathology Post Midterm (50 decks)

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