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Pathology 1 Block 3 > WBC & LN: Part 4: MDS and Myeloproliferative Disorders > Flashcards

WBC & LN: Part 4: MDS and Myeloproliferative Disorders Flashcards

1
Q

what is Myelodysplastic Syndrome?

it has an increased risk for what?

it usually presents how?

A

group of ‘clonal stem cell’ disorders characterized by maturation defects associated with ineffective hematopoiesis

increased risk of cytopenias, and transformation to acute myelogenous leukemia (AML)

presents as peripheral blood cytopenias (fall in total counts) as a result of exaggerated apoptosis of marrow precursor cells

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2
Q

On what part of the population does MDS afect?

what may cause it?

A

older age group (exception: following chemotherapy/associated with Down’s Syndrome)

causes

  • Chemicals: benzene, alkylating agents
  • radiation
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3
Q

what are the 2 types of MDS?

what is the pathogenesis of MDS?

A
  1. Idiopathic Primary MDS
  2. Therapy related MDS (t-MDS)
  • ineffective hematopoiesis
  • possibly due to stem cell damage
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4
Q

what can be expected in a lab report of a patient with MDS?

Peripheral Blood smear will show what?

A

pancytopenia, neutropenia, thrombocytopenia

Peripheral Blood Smear = hypogranular granulocytes, pseudo Pelger-Huet cells

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5
Q

what is this?

A

MDS: peripheral blood smear showing ‘bilobed’ (pseudo Pelger Huet) neutrophils

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6
Q

What can we find out by looking at a Bone Marrow with Myelodysplastic Syndrome?

how much myeloblasts will be seen in the bone marrow?

A
  • dysplastic maturation affecting all non lymphoid lineage: erythroid, granulocytic, monocytic, megakaryocyctic
  • Erythroid series: ringed sideroblasts, megaloblastoid cells
  • Myeloid series: pseudo Pelger-Huet cells; neutrophils with 2 nuclear lobes
  • Megakaryocyte: pawn ball megakaryocytes (single nuclear lobes)
    • myeloblasts less than 20% in bone marrow
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7
Q

what is this?

A

MDS: bone marrow showing ‘pawn ball’ megakaryocytes

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8
Q

What age group will myelodysplastic syndrome affect?

what will be the clinical presentation?

what can MDS progress into?

what is the prognosis for MDS?

A
  • older age group (> 60 years)
  • weakness, infections, hemorrhages
  • progression to AML in 10 to 40%
  • t- MDS has very poor prognosis
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9
Q

what is this?

What disease is it related to?

A

ringed sideroblasts

MDS

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10
Q

what is this?

What disease is it related to?

A

pseudo Pelger-Huet cells

MDS

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11
Q

what is this?

What disease is it related to?

A

Pawn ball megakaryocyte

MDS

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12
Q

Myeloproliferative disorders

what characterizes myeloproliferative disorders?

how do you classify MPD?

what are the common features we can expect to see in MPD?

A
  • neoplastic cell proliferation associated with cell maturation involving all cell lines
  • classified on the predominant cell type affected
  1. association with JAK-2 mutation
  2. increased cell turnover (exception ET)
  3. marrow fibrosis (exception ET)
  4. transformation to acute leukemias, mostly AML, but some end with ALL (transformation to acute leukemia not seen with ET)
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13
Q

Myeloproliferative Disorders

what are the 4 types of MPD?

A
  1. Chronic Myeloid Leukemia (CML)
  2. Polycythemia vera
  3. Essential thrombocytosis
  4. Primary myelofibrosis
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14
Q

Chronic Myeloid Leukemia

who does it affect?

what is the Pathophysiology involved?

A
  • adults between 25 to 60 yrs
  • presence of a distinctive molecular abnormality

translocation involving ABL gene on chromosome 9 and BCR gene on chromosome 22 (philadelphia ch.)

activates multiple downstream pathways: RAS, JAK/STAT, AKT

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15
Q

Labs for Chronic Myeloid Leukemia

total WBC?

Platelet count?

what can be seen in the Peripheral Blood Smear?

Bone Marrow?

Biochemistry?

Molecular Diagnosis?

A

total WBC = more than 100, 000

Platelet count = increased first, later thrombocytopenia

Peripheral Blood Smear = moderate anemia, most cells present are:

myelocytes, metamyelocytes, band forms, eosinophils and basophils

Bone Marrow =

  • marked hypercellularity
  • myeloid and megakaryocytic lineage
  • eosinophils and basophils are increased
  • myeloblasts are less than 5% (increase over 10% signifies onset of accelerated phase)
  • later stages show collagen proliferation

Biochemistry =

  • hyperuricemia
  • LAP score is low

Molecular Diagnosis = Philadelphia chromosome (95%)

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16
Q

what is seen here?

A

Chronic Myeloid Leukemia

Note: numerous granulocytes; mature neutrophils seen, and numerous platelets

17
Q

what disease is this?

what can be seen in this bone marrow aspiration?

A

CML

increase in myeloid elements including basophils and eosinophils.

18
Q

What are the clinical signs of CML?

during what stage do we see enlarged spleen?

A
  • massive splenomegaly
  • rapidly increasing percentage of blasts (myeloblasts)
  • anemia, thrombocytopenia, basophilia

enlarged spleen is seen during the blast crisis or accelerated phase.

19
Q

Myeloblasts in blast crisis or accelerated phase lack what?

What is used to treat CML? what does it do?

A

Auer rods

Imatinib mesylate; blocks the effects of the BCR-ABL fusion product

20
Q

on who does polycythemia vera happen?

what is a cause that leads to polycythemia vera?

Reactive Polycythemia vera is associated with what? What are the clinical findings?

Ectopic Polycythemia vera is associated with what? what are the clinical findings?

what is seen in a lab report for Polycythemia Vera?

A

adults

neoplasm arising in a multipotent myeloid stem cell

lung diseases; Sa O2 is low, and EPO is high

paraneoplastic syndromes like renal cell carcinoma; SaO2 is normal, EPO is high

SaO2 is normal, and EPO is decreased

21
Q

what characterizes polycythemia vera?

what is the pathogenesis of polycythemia vera?

A

characterized by increased marrow production of: erythroid, granulocytic and megakaryocytic elements

  • erythroid proliferation not regulated by erythropoietin due to decreased erythropoietin levels; the little EPO present drives red cell expansion as erythrocytes are sensitive to erythropoietin
  • JAK2 mutation in chromosome 9
    • gets activated and causes proliferation
22
Q

what can be expected for the following values during polycythemia vera?

RBC count?

Hb?

Hematocrit?

WBC?

platelets?

bone marrow?

peripheral blood smear?

biochemical?

A

RBC count = increased

Hb = increased

Hematocrit = increased

WBC = leukocytosis

platelets = increased (thrombocytopenia)

bone marrow = hypercellular (late stage = fibrosis)

peripheral blood smear = increased basophils & large platelets

biochemical = hyperuricemia

23
Q

why do most clinical features in polycythemia vera happen?

what are the clinical features of polycythemia vera?

A
  • mostly due to expanded total blood volume and slowing of blood flow due to increased viscosity

C/F:

  • headache, dizziness, tinnitus, visual disturbances
  • “ruddy” color of skin, especially face
  • DVT, infarcts, and bleeding (increased red cell mass, and abnormal platelet function)
  • transient ischemic attack, stroke, pulmonary thrombo-embolism, Budd-Chiari syndrome
  • pruritus (release of histamine from mast cells/basophils), ‘aquagenic pruritus’
  • peptic ulceration (histamine release)
  • hyperuricemia (gout)
24
Q

what is a complication of polycythemia vera?

A

myelofibrosis, acute leukemia

25
Q

what is primary myelofibrosis?

what is its pathogenesis?

A

a disease of adults (over 60 years), presenting with anemia, bone marrow fibrosis and splenomegaly

pathogenesis:

  • extensive deposition of collagen (cause of fibrosis)
  • fibrogenic cytokines (factors): PDGF and TGF-β

released from neoplastic megakaryocytes

  • JAK-2 mutation
  • extramedullary hematopoiesis (spleen and liver)
26
Q

what can we expect for these values in primary myelofibrosis:

Hemoglobin?

WBC count?

Peripheral blood smear?

Bone Marrow?

Biochemistry?

A

Hemoglobin = reduced

WBC count = elevated in early stage

Peripheral blood smear = normocytic normochromic anemia and tear drop’ rbc (dacrocytes) + leukoerythroblastic reaction

Bone Marrow = early: hypercellular; later: hypocellular

Biochemistry = hyperuricemia

27
Q

what disease is this?

what are the cells in the bottom?

what is the cell on the right?

A

primary myelofibrosis

tear drop cells RBC

nucelated RBC

28
Q

what is this?

A

fibrosis in the bone marrow

29
Q

Essential Thrombocytosis

how common is it, among the myeloproliferative disorders?

what is essential thrombocytosis?

it is associated with what complications?

what is the pathogenesis of this condition?

what differs essential thrombocytosis from polycythemia or marrow fibrosis?

A

uncommon

is a clonal hematopoietic stem cell disorder characterized by an isolated thrombocytosis and

associated with thrombotic and hemorrhagic complications

pathogenesis:

  • JAK2 mutation
  • leads to proliferation of platelets

no evidence of polycythemia, nor marrow fibrosis seen in ET

30
Q

what can we expect for these values for essential thrombocytosis:

total WBC

total platelet count

peripheral smear

bone marrow

bleeding time

platelet defect

A

total WBC = elevated

total platelet count = elevated

peripheral smear = anemia, basophilia, leukocytosis, thrombocytosis (increased number), platelets are larger

bone marrow = increased cellularity, megakaryocytic hyperplasia

bleeding time = may be abnormal

platelet defect = present

31
Q

what can be seen in this peripheral smear?

In what disease do we see this?

A

platelets increased

seen in essential thrombocytosis

32
Q

what are the clinical features for essential thrombocytosis?

what can induce the symptoms?

A
  • deep vein thrombosis, portal and hepatic vein thrombosis
  • myocardial infarction
  • tendency to bleed, GI bleed
  • splenomegaly
  • Erythromyalgia ( intense burning or throbbing pain of hand and feet)

****associated with warmth, duskiness, and mottled erythemia

—— induced by exercise ——

Pathology 1 Block 3 (7 decks)

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