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White blood cell disorders Flashcards

1
Q

What is the starting cell for hematopoiesis?

A
  • CD-34+ hematopoietic stem cells
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2
Q

What are the causes of neutropenia?

A
  • Drug - especially chemotherapy alkylating agents–> decrease in WBC count especially neutrophils.
  • Infection leading to aggregation of neutrophils in the tissue–> decrease in circulating neutrophils
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3
Q

What are the causes of leukopenia?

A
  • Immunodeficiency (DiGeorge or HIV)
  • Autoimmune destruction (SLE)
  • High cortisol (exogenous or Cushings)
  • Whole body radiation
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4
Q

What is the pathogenesis of acute leukemia?

A
  • Increased production of blast cells crowds out normal hematopoiesis leading to anemia, thrombocytopenia and neutropenia.
  • Elevated levels of immature blast (large, immature with punched out nuclei.
  • Type depends on the phenotype of the blast.
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5
Q

What are the two types acute lymphocytic leukemia (ALL) and their markers?

A
  • B-ALL - most common TDT+, CD-10,19,20

- T-ALL - TDT+, CD-2-8, no CD-10

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6
Q

How is lymphoblastic leukemia differentiated from myeloblastic (marker)?

A
  • TdT+
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7
Q

What is the epidemiology of ALL?

A
  • Typically arise in children, down syndrome is a risk factor.
  • T-ALL arises in teenagers.
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8
Q

What abnormalities are associated with B-ALL? What are their prognosis?

A
  • t(12,21) - children, good prognosis, chemo, prophylaxis to scrotum and CNS.
  • t(9,22) - PH chromosome, adult, poor prognosis.
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9
Q

How do teenagers present with T-ALL?

A
  • Mediastinal (thymic) mass.
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10
Q

How is acute myeloid leukemia (AML) identified?

A
  • Myeloperoxidase (MPO) which may seen as Auer rods.
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11
Q

What are the high yield subtypes of AML?

A
  • Promyelocytic
  • Monocytic
  • Megakaryocytic
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12
Q

Describe acute promyelocytic leukemia

A
  • t(15;17) Retinoic acid R (RAR) translocation 17–>15. Disruption blocks maturation and cells build up>
  • Immature promyelocytes have increased primary granules–> increased risk for DIC
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13
Q

How is acute promyelocytic leukemia treated?

A
  • Treatment with all- trans- retinoic acid (ATRA)–> binds altered receptor causing cells to mature.
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14
Q

Describe acute monocytic leukemia

A
  • Increase in monocytes, MPO negative

- Characteristically infiltrates gums.

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15
Q

Describe acute megakaryocytic leukemia?

A
  • Increased megakaryocytes, MPO negative

- Associated with down syndrome arises before the age of 5.

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16
Q

What are the risk factors for AML?

A
  • May arise from pre-existing dysplasias (Myelodysplasic syndromes) especially with alkylating agents and radiation therapy.
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17
Q

How do Myelodysplasic syndromes usually present and how do they die?

A
  • Cytopenias, hypercelluar bone marrow, increased blasts
    and abnormal maturation cells.
  • Most die from bleeding or infection but some progress to acute leukemia
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18
Q

What is the pathogenesis of chronic leukemia?

A
  • Neoplastic proliferation of mature circulating lymphocytes

- Usually insidious onset in older adults.

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19
Q

What are the characteristics of chronic lymphocytic leukemia (CLL)?

A
  • The most common leukemia in US
  • Increased proliferation of naive B cells co-expressing CD5 and 20
  • Increased lymphocytes and smudge cells on blood smear
  • Involvement of lymph nodes leads to lymphadenopathy and is called small cell lymphoma
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20
Q

What are the complications with CLL?

A
  • Hypogammaglobulinemia- infection is the most common cause of death
  • Autoimmune hemolytic anemia
  • (Richter) transformation to diffuse large B cell lymphoma, enlarged lymph nodes and spleen.
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21
Q

What are the characteristics of hairy cell leukemia?

A
  • Chronic leukemia
  • Neoplastic proliferation of mature B cells characterized by hairy cytoplasmic process.
  • Cells are positive for tartrate-resistant acid phosphatase (TRAP)
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22
Q

How do patients present with hairy cell leukemia?

A
  • No lymphadenopathy
  • Splenomegaly (infiltration of red pulp)
  • Dry tap on bone marrow aspiration due to marrow fibrosis.
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23
Q

How are patients treated with hairy cell leukemia?

A
  • Patients respond to 2-CDA (cladribine), adenosine deaminase inhibitor, adenosine accumulates to toxic levels in neoplastic B cells.
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24
Q

What is Adult T cell leukemia/ lymphoma (ATLL)?

A
  • Chronic leukemia

- Neoplastic proliferation of mature CD4+ T cells

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25
What is ATLL associated with and how does it present?
- HTLV-1, japan and caribean - Rash - Lymphadenopathy and hepatosplenomegaly - Lytic bone lesions with hypercalcemia
26
What is mycosis fungoides?
- Chronic leukemia - Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin producing rash, plaques and nodules (Pautrier microabscesses)
27
What occurs when mycosis fungoides infiltrates the blood?
- Sezary syndrome | - Lymphocytes with cerebriform nuclei
28
What are the myeloproliferative disorders?
- Chronic myeloid leukemia (CML) - Polycythemia Vera - Essential thrombocytopenia - Myelofibrosis
29
What are the complications with myeloproliferative disorders?
- Increased risk for hyperuricemia and gout due to high cell turnover (nucleotides--> uric acid) - Progression to bone marrow fibrosis or transformation to acute leukemia.
30
What is the pathogenesis of chronic myeloid leukemia?
- Neoplastic proliferation of mature myeloid cells, especially granulocytes, elevated basophils are characteristic. - Caused by t(9,22) Philly chromosome--> BCR-ABL fusion protein with increased tyrosine kinase activity.
31
How does CML present?
- Most often asymptomatic - Patients can present with splenomegaly in accelerated phase of disease, transformation to acute leukemia usually follows - AML or ALL due to mutation in pluripotent stem cell
32
How is CML treated/
- Imatinib (Gleevec) | - Being replaced by dasatinib and nilotinib
33
How is CML distinguished fro leukemoid rxn?
- Negative leukocyte alkyline phosphatase (LAP) - Philly chromosome - Increased basophils
34
What is the pathogenesis of polycythemia vera?
- Neoplastic proliferation of mature myeloid cells especially RBC - Granulocytes and platelets as well - Associated with JAK 2 kinase mutation
35
What are the clinical symptoms of polycythemia vera?
- Related to hyperviscosity - Blurred vision, HA - Flushed face - Itchy skin especially after bathing (due to histamine release from increased mast cells)
36
How is polycythemia vera treated?
- Phlebotomy, second line treatment hydroxyurea (ribonucleotide reductase inhibitor) - Without treatment death usually occurs within one year.
37
How is polycythemia vera distinguished from reactive polycythemia?
- In PV EPO levels are decreased and SaO2 levels are normal. - In RP due to high altitude EPO is increased and SaO2 is low. - In RP due to renal cell carcinoma, EPO is increased and SaO2 is normal.
38
What is the pathology of essential thrombocythemia?
- Neoplastic proliferation of mature of mature myeloid cells especially platelets - Granulocytes and RBC's also increased. - Associated with JAK 2 kinase mutation.
39
What are the symptoms of essential thrombocythemia?
- Increased bleeding or thrombosis - Rarely progresses to marrow fibrosis or acute leukemia - No significant risk of hyperuricemia or gout
40
What is the pathogenesis of myelofibrosis?
- Neoplastic proliferation of mature myeloid cells especially megakaryocytes resulting in excess platelet derived growth factor (PDGF) and marrow fibrosis. - Associated with JAK 2 kinase mutation
41
What are the clinical features of myelofibrosis?
- Splenomegaly due to extramedullary hematopoiesis and increased risk of infection, thrombus, or bleeding - Leukooerythroblastic smear (tear-drop RBC, nuleated RBC and immature granulocytes)
42
When is painful lymphadenopathy seen?
- In nodes that are draining a region of infection
43
When is painless lymphadenopathy seen?
- In chronic inflammation (chronic lymphadenopathy, metastatic carcinoma, lymphoma)
44
What region of lymphadenopathy is seen in rheumatoid arthritis and early stages of HIV?
- Follicular hyperplasia (B-cell)
45
What region of lymphadenopathy is seen with viral infections like infectious mononucleosis?
- Paracortex hyperplasia (T-cell)
46
What region of lymphadenopathy is seen in nodes draining a region of cancer?
- Hyperplasia of sinus histiocytes
47
What is follicular lymphoma and its mechanism?
- Neoplastic proliferation of small B-cells(CD-20) that produce follicle like nodules. - t(14;18) BLC2 translocates from 18 to 14 to Ig heavy chain locus - Over expression of BCL2 inhibits apoptosis
48
How does follicular lymphoma normally present and what are the complications?
- Late adulthood with painless lymphadenopathy | - Can progress to diffuse large B-cell lymphoma, when patient presents with an enlarging lymph node
49
How is follicular lymphoma distinguished from reactive follicular hyperplasia?
- Disruption of normal lymph node architecture - Lack of tingible body macrophages in germinal centers - BCL2 expression in follicles - Monoclonality
50
What is the treatment for follicular lymphoma?
- Reserved for patients that are symptomatic, low-dose chemotherapy or Rituximab (anti CD20 antibody)
51
What are the non-hodgkins lymphomas?
- Follicular lymphoma - Mantle cell lymphoma - Diffuse Large B-cell lymphoma - Marginal cell lymphoma - Burkitt lymphoma
52
What is marginal zone lymphoma?
- Neoplastic proliferation of small B cells (CD-20) that expands into the marginal zone.
53
What is associated with Marginal zone lymphoma?
- Chronic inflammatory states like Hashimoto's thyroiditid, Sjogren syndrome, and H. Pylorii - MALToma is marginal zone lymphoma in mucosal sites
54
What is Burkitt lymphoma?
- Neoplastic proliferation of intermediate B-cells associated with EBV
55
How does Burkitt lymphoma present?
- Presents as an extranodal mass in child or young adult - African form usually involves the Jaw - Sporadic form usually involves the abdomen - High mitotic index and starry sky appearance on microscopy
56
What is the mechanism of burkitt lymphoma?
- t(8,14) of c-myc 8 to 14 Ig heavy chain locus
57
What is diffuse large B-cell lymphoma?
- Neoplastic proliferation of large B cells that grow diffusely in large sheets - Highly aggressive - Most common form of non-hodgkins lymphoma
58
What causes large B cell lymphoma and how does it present?
- Arises sporadically of transformation from low grade lymphoma (follicular lymphoma) - Late adult hood with enlarging lymph node or extranodal mass
59
What is Hodgkins lymphoma?
- Neoplastic proliferation of Reed-Sternberg (RS) cells, which are large B cells with multilobed nuclei and prominent nucleoli (owl-eyed nuclei); classically positive for CD15 and CD30
60
What is the effect of Reed-sternburg cells?
- Secrete cytokines: "B" symptoms (fever,chills, night sweats); Attract reactive lymphocytes, plasma cells, macrophages and eosinophils; May result in fibrosis
61
What are the different subtypes of Hodgkins lymphoma?
- Nodular sclerosis - Lymphocyte rich - Mixed cellularity - Lymphocyte depleted
62
What is the most common subtype of Hodgkins lymphoma?
- Nodular sclerosis (70%)
63
How does nodular sclerosis present?
- Enlarged cervical or mediastinal lymph nodes, usually female - Lymph node divided by bands of sclerosis, RS cells present in lake like spaces
64
Which form of Hodgkins lymphoma has the best prognosis?
- Lymphocyte rich
65
What is associated with Mixed cellularity?
- Abundant eosinophils (RS cells produce IL-5)
66
Which form of Hodgkins lymphoma is the most aggressive?
- Lymphocyte depleted
67
What are the different plasma cell disorders (dyscrasias)
- Multiple myeloma - Monoclonal gammopathy of undetermined significance (MGUS) - Waldenstrom macroglobulinemia
68
What is multiple myeloma?
- Proliferation of plasma cells in the bone marrow - Most common primary malignancy of bone - High IL-6 is present stimulating plasma cell proliferation and immunoglobulin production
69
What are the clinical features of multiple myeloma?
- Bone pain - Hypercalcemia - Rouleaux formation - Increased risk of infection - Increased serum protein, primary AL amyloidosis (free light chains deposit in the tissue) and proteinuria
70
What is Monoclonal gammopathy of undetermined significance (MGUS)?
- Increased serum M spike with other features of multiple myeloma absent - Typically occurs in the elderly - Few cases develop into multiple myeloma
71
What is Waldenstrom macroglobulinemia?
- B-cell lymphoma with monoclonal IgM production
72
What are the clinical features of Waldenstrom macroglobulinemia?
- Generilized lymphadenopathy without bone lesions - Increased serum protein with M spike - Visual and neurologic deficits (retinal hemorrhage or stroke) - Hyper viscosity--> defective platelet aggregation--> bleeding
73
How is Waldenstrom macroglobulinemia treated?
- Plasmapheresis which removes IgM

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