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Bood and Lymph > White blood cell disorders > Flashcards

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
Q

What is ATLL associated with and how does it present?

A
  • HTLV-1, japan and caribean
  • Rash
  • Lymphadenopathy and hepatosplenomegaly
  • Lytic bone lesions with hypercalcemia
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26
Q

What is mycosis fungoides?

A
  • Chronic leukemia
  • Neoplastic proliferation of mature CD4+ T cells that infiltrate the skin producing rash, plaques and nodules (Pautrier microabscesses)
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27
Q

What occurs when mycosis fungoides infiltrates the blood?

A
  • Sezary syndrome

- Lymphocytes with cerebriform nuclei

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28
Q

What are the myeloproliferative disorders?

A
  • Chronic myeloid leukemia (CML)
  • Polycythemia Vera
  • Essential thrombocytopenia
  • Myelofibrosis
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29
Q

What are the complications with myeloproliferative disorders?

A
  • Increased risk for hyperuricemia and gout due to high cell turnover (nucleotides–> uric acid)
  • Progression to bone marrow fibrosis or transformation to acute leukemia.
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30
Q

What is the pathogenesis of chronic myeloid leukemia?

A
  • 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
Q

How does CML present?

A
  • 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
Q

How is CML treated/

A
  • Imatinib (Gleevec)

- Being replaced by dasatinib and nilotinib

33
Q

How is CML distinguished fro leukemoid rxn?

A
  • Negative leukocyte alkyline phosphatase (LAP)
  • Philly chromosome
  • Increased basophils
34
Q

What is the pathogenesis of polycythemia vera?

A
  • Neoplastic proliferation of mature myeloid cells especially RBC
  • Granulocytes and platelets as well
  • Associated with JAK 2 kinase mutation
35
Q

What are the clinical symptoms of polycythemia vera?

A
  • Related to hyperviscosity
  • Blurred vision, HA
  • Flushed face
  • Itchy skin especially after bathing (due to histamine release from increased mast cells)
36
Q

How is polycythemia vera treated?

A
  • Phlebotomy, second line treatment hydroxyurea (ribonucleotide reductase inhibitor)
  • Without treatment death usually occurs within one year.
37
Q

How is polycythemia vera distinguished from reactive polycythemia?

A
  • 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
Q

What is the pathology of essential thrombocythemia?

A
  • Neoplastic proliferation of mature of mature myeloid cells especially platelets
  • Granulocytes and RBC’s also increased.
  • Associated with JAK 2 kinase mutation.
39
Q

What are the symptoms of essential thrombocythemia?

A
  • Increased bleeding or thrombosis
  • Rarely progresses to marrow fibrosis or acute leukemia
  • No significant risk of hyperuricemia or gout
40
Q

What is the pathogenesis of myelofibrosis?

A
  • 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
Q

What are the clinical features of myelofibrosis?

A
  • Splenomegaly due to extramedullary hematopoiesis and increased risk of infection, thrombus, or bleeding
  • Leukooerythroblastic smear (tear-drop RBC, nuleated RBC and immature granulocytes)
42
Q

When is painful lymphadenopathy seen?

A
  • In nodes that are draining a region of infection
43
Q

When is painless lymphadenopathy seen?

A
  • In chronic inflammation (chronic lymphadenopathy, metastatic carcinoma, lymphoma)
44
Q

What region of lymphadenopathy is seen in rheumatoid arthritis and early stages of HIV?

A
  • Follicular hyperplasia (B-cell)
45
Q

What region of lymphadenopathy is seen with viral infections like infectious mononucleosis?

A
  • Paracortex hyperplasia (T-cell)
46
Q

What region of lymphadenopathy is seen in nodes draining a region of cancer?

A
  • Hyperplasia of sinus histiocytes
47
Q

What is follicular lymphoma and its mechanism?

A
  • 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
Q

How does follicular lymphoma normally present and what are the complications?

A
  • Late adulthood with painless lymphadenopathy

- Can progress to diffuse large B-cell lymphoma, when patient presents with an enlarging lymph node

49
Q

How is follicular lymphoma distinguished from reactive follicular hyperplasia?

A
  • Disruption of normal lymph node architecture
  • Lack of tingible body macrophages in germinal centers
  • BCL2 expression in follicles
  • Monoclonality
50
Q

What is the treatment for follicular lymphoma?

A
  • Reserved for patients that are symptomatic, low-dose chemotherapy or Rituximab (anti CD20 antibody)
51
Q

What are the non-hodgkins lymphomas?

A
  • Follicular lymphoma
  • Mantle cell lymphoma
  • Diffuse Large B-cell lymphoma
  • Marginal cell lymphoma
  • Burkitt lymphoma
52
Q

What is marginal zone lymphoma?

A
  • Neoplastic proliferation of small B cells (CD-20) that expands into the marginal zone.
53
Q

What is associated with Marginal zone lymphoma?

A
  • Chronic inflammatory states like Hashimoto’s thyroiditid, Sjogren syndrome, and H. Pylorii
  • MALToma is marginal zone lymphoma in mucosal sites
54
Q

What is Burkitt lymphoma?

A
  • Neoplastic proliferation of intermediate B-cells associated with EBV
55
Q

How does Burkitt lymphoma present?

A
  • 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
Q

What is the mechanism of burkitt lymphoma?

A
  • t(8,14) of c-myc 8 to 14 Ig heavy chain locus
57
Q

What is diffuse large B-cell lymphoma?

A
  • Neoplastic proliferation of large B cells that grow diffusely in large sheets
  • Highly aggressive
  • Most common form of non-hodgkins lymphoma
58
Q

What causes large B cell lymphoma and how does it present?

A
  • Arises sporadically of transformation from low grade lymphoma (follicular lymphoma)
  • Late adult hood with enlarging lymph node or extranodal mass
59
Q

What is Hodgkins lymphoma?

A
  • 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
Q

What is the effect of Reed-sternburg cells?

A
  • Secrete cytokines: “B” symptoms (fever,chills, night sweats); Attract reactive lymphocytes, plasma cells, macrophages and eosinophils; May result in fibrosis
61
Q

What are the different subtypes of Hodgkins lymphoma?

A
  • Nodular sclerosis
  • Lymphocyte rich
  • Mixed cellularity
  • Lymphocyte depleted
62
Q

What is the most common subtype of Hodgkins lymphoma?

A
  • Nodular sclerosis (70%)
63
Q

How does nodular sclerosis present?

A
  • Enlarged cervical or mediastinal lymph nodes, usually female
  • Lymph node divided by bands of sclerosis, RS cells present in lake like spaces
64
Q

Which form of Hodgkins lymphoma has the best prognosis?

A
  • Lymphocyte rich
65
Q

What is associated with Mixed cellularity?

A
  • Abundant eosinophils (RS cells produce IL-5)
66
Q

Which form of Hodgkins lymphoma is the most aggressive?

A
  • Lymphocyte depleted
67
Q

What are the different plasma cell disorders (dyscrasias)

A
  • Multiple myeloma
  • Monoclonal gammopathy of undetermined significance (MGUS)
  • Waldenstrom macroglobulinemia
68
Q

What is multiple myeloma?

A
  • 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
Q

What are the clinical features of multiple myeloma?

A
  • 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
Q

What is Monoclonal gammopathy of undetermined significance (MGUS)?

A
  • Increased serum M spike with other features of multiple myeloma absent
  • Typically occurs in the elderly
  • Few cases develop into multiple myeloma
71
Q

What is Waldenstrom macroglobulinemia?

A
  • B-cell lymphoma with monoclonal IgM production
72
Q

What are the clinical features of Waldenstrom macroglobulinemia?

A
  • 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
Q

How is Waldenstrom macroglobulinemia treated?

A
  • Plasmapheresis which removes IgM

Bood and Lymph (8 decks)

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