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Pathology II Exam 3 > 2 hematopathology 1: Myeloid and Lymphoid Bone Marrow Disorders (mike) > Flashcards

2 hematopathology 1: Myeloid and Lymphoid Bone Marrow Disorders (mike) Flashcards

1
Q

What is a chronic myeloproliferative disorder with increased neutrophils and granulocytic precursors? Is there an increase in basophils? Is it common in children?

A
  1. CML
  2. Yes, this is a key characteristic
  3. No- and its only 15% of leukemias in adults
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2
Q

What are some possible symptoms of CML?

A

lethargy, fatigue, weight loss, splenomegaly

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3
Q

What do we see in the peripheral blood in CML? What about the bone marrow?

A
  1. leukocytosis, left shift with full range of myeloid progenitors, ABSOLUTE BASOPHILIA.
  2. hyper cellular with increased myeloid:erythroid ratio
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4
Q

What do the megakaryocytes look like in CML?

A

small hypolobate

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5
Q

Shotened chromosome 22 is known as the what?

A

philadelphia chromosome

t(9;22)

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6
Q

What type of enzyme is ABL? what happens when it is fused to BCR? what do we treat with?

A
  1. Tyrosine Kinase
  2. enzyme becomes constitutively activated–> downstream proliferation and differentiation
  3. Imatinib
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7
Q

Since new drug findings, what is the current prognosis of CML?

A

full life expectancy

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8
Q

what is a clonal lymphoproliferative disorder of mature lymphocytes?

A

CLL

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9
Q

What is expressed in CLL?B-

A

B-cell neoplasms which express mature B-cell antigens, monotypic kappa or lambda light chains, and ABERRANTLY EXPRESS CD5.

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10
Q

Is CLL indolent or aggressive? is it more common in males or females?

A
  1. Indolent

2. males

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11
Q

Is generalized lymphadenopathy common in CLL?

A

50-60% will have along with lymphocytosis, liver and spleen involvement

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12
Q

What cells do we see on a peripheral blood smear of CLL patient?

A
  1. monomorphous small mature lymphocytes with clumped chromatin
  2. smudge cells
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13
Q

Bone marrow biopsy in CLL will show nodular, interstitial or diffuse involvement by what cells?

A

small mature lymphocytes with predominantly round nuclei.

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14
Q

What is a clonal myeloid stem cell disorder with cytopenias, ineffective hematopoiesis, and dyspoiesis? what is the increased risk of transformation to in this case?

A
  1. myelodysplastic syndromes

2. AML

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15
Q

What is abnormal morphologic features in the maturing myeloid, erythroid and megakaryocytic cells?

A
  1. Dyspoiesis
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16
Q

How do people present with Myelodysplastic Syndromes? what do 1/3 of patients progress to?

A
  1. 50% with infections, hemorrhage and fatigue and 50% asymptomatic
  2. acute leukemia
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17
Q

Review the WHO classification of MDS-

A

Classification based on
–Number of blasts in peripheral blood and bone marrow
–Cytologic features•Ring sideroblasts•Dyspoiesis in one, two or all three cell lines
–Cytogenetic abnormalities

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18
Q

What is the morphology of MDS?

A

Pancytopenia with dyspoietic features of RBCs, granulocytes, platelets/megak.

you may also see increased myeloblasts

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19
Q

Dimorphic red cells is very prominent in MDS blood smears- what are the two distinctly different morphologies?

A
  1. normochromic normocytic

2. hypochromic microcytic

20
Q

What is dysgranulopoiesis?

A

hypersegmented neutrophils, hypolobate, hypogranular

should be able to compare with normal neutrophils on same slide to ensure that it is not just a poor stain

21
Q

Review the types of dyserythropoiesis?

A

nuclear budding, nuclear irregularity, megaloblastic change (nuclear to cytoplasmic dyssynchrony), multinucleation, ring sideroblasts

22
Q

In dysmegakaryopoiesis , what does the megakaryocytic look like?

A

small hypolobate or widely spaced nuclear lobes

23
Q

Are specific translocations common in MDS?

A

No- trisomies and deletions are common

24
Q

What are clonal proliferations of multi potent myeloid stem cells resulting in an increased number of differentiated granulocytes, RBCs or platelets?

A

Chronic myeloproliferative neoplasms- The disorders have an acquired defect of the proliferative control of the stem cell.

25
Q

What are the 4 types of chronic myeloproliferative neoplasms?

A
  1. CML
  2. polycythemia vera
  3. essential thrombocythemia
  4. primary myelofibrosis
26
Q

what major mutation leads to increased proliferation of bone marrow cells?

A

JAK2 V617F

most patients with polycythemia vera and approximately half the patients with essential thrombocythemia or primary mrelofibrosis

27
Q

What is chronic myeloproliferative disorder associated with thrombocytosis and increased megakaryocytes in the bone marrow.?

A

Essential thrombocythemia-

Platelet counts can range from slightly above normal to several million/ul.

28
Q

How does essential thrombocythemia present?

A

thrombosis or hemorrhage, splenomegaly in 50%

29
Q

How does essential thrombocythemia present?
what does a blood smear show?
what does a bone marrow aspirate show?

A
  1. thrombosis or hemorrhage, splenomegaly in 50%
  2. increased an large platelets
  3. very large and atypical megakaryocyte
30
Q

what is clonal myeloproliferative neoplasm characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that in fully developed disease is associated with reactive deposition of fibrous connective tissue and with extramedullary hematopoiesis?

A

primary myelofibrosis

progressive splenomegaly
median survival=3 years
5-20% progress to leukemia

31
Q

What is the blood morphology of primary myelofibrosis?

A
  1. anemia with teardrop red blood cells

2. leukoerythroblastic reaction- immature myeloid cells and nucleated RBC circulating

32
Q

What are the two types of bone marrow morphology found in primary myelofibrosis?

A
  1. cellular stage with up neutrophils and clusters of atypical megakaryocytes
  2. fibrotic stage- with intrasinusoidal hematopoiesis
33
Q

What type of appearance does the bone marrow have in fibrotic star of primary myelofibrosis?

A

has streaming appearance due to reticulin fibrosis

34
Q

What is an important gross anatomical feature of primary myelofibrosis?

A

marked splenomegaly- extra medullary hematopoiesis is taking place

35
Q

What is a clonal proliferation of terminally differentiated B cells capable of secreting immunoglobulin or portions of immunoglobulin? what is the monoclonal immunoglobulin called?

A
  1. plasma cell neoplasms

2. M-protein or monoclonal protein- seen on serum protein electrophoresis

36
Q

Review the 3 types of plasma cell neoplasms

A

Monoclonal gammopathy of undetermined significance
plasmacytoma
plasma cell myeloma

37
Q

What is characterized by a solitary bone lesion or extra osseous lesion> what are the common extra osseous sites? How do we treat?

A
  1. solitary myeloma-plasmacytoma
  2. lung, pharynx, nasal sinuses
  3. radiation
38
Q

Where does plasma cell myeloma originate?

A

bone marrow- involvement of the skeleton at multiple sites

39
Q

What are the symptoms for plasma cell myeloma?

A 
CRAB
hyper calcemia
renal insufficiency
Anemia/Amyloid symptoms
bony lytic lesions

ALSO- RECURRENT INFECTIONS

40
Q

In asymptomatic plasma cell myeloma, what are we typically looking for?

A

significant M-protein in serum

significant bone marrow involvement

41
Q

What is a classic finding in plasma cell myeloma on blood smear?

A

Rouleaux formation- linear arrays of red cells

42
Q

T-F-Plasma cells in myeloma may be either mature/normal or immature?

A

True

43
Q

what are the features of mature normal plasma cell morphology?

A

eccentric nuclei
perinuclear hof/golgi clearing
clumped chromatin
mod. abundant cytoplasm

44
Q

What are the features of abnormal/immature plasma cells?

A

nuclear enlargement

prominent nucleoli

45
Q

Is treatment of myeloma curative?

A

No

46
Q

What general classes of drugs have increased survival in multiple myeloma from 3 to >8 years?

A
  1. immune modulators
  2. proteasome inhibitors
  3. autologous stem cell transplant tech too.

Pathology II Exam 3 (13 decks)

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