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Pathophysiology - Hematology > Malignant Lymphocyte Disorders > Flashcards

Malignant Lymphocyte Disorders Flashcards

1
Q

What are the important chronic lymphoid leukemias?

A
  1. Chronic lymphocytic leukemia (CLL)
  2. Prolymphocytic leukemia (PLL)
  3. Hairy cell leukemia (HCL)
  4. Large granular lymphocytic leukemia (LGLL)
  5. Adult T cell leukemia/lymphoma (ATLL)
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2
Q

Who tends to get chronic lymphoid leukemias and what are the most typical clinical features?

A

Older adults, diseases have indolent course (except ATLL)

Lymphadenopathy, hepatosplenomegaly (like ALL)

Constitutional symptoms - fever, night sweats, weight loss, fatigue

Abnormal CBC with bone marrow infiltration

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3
Q

What is the cell morphology of chronic lymphocytic leukemia (CLL)?

A
  1. Small, mature-looking lymphocytes indistinguishable from normal
  2. Smudge cells present - ruptured lymphocytes (cells are fragile in CLL) CLL = crushed little lymphocytes
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4
Q

What is the most common leukemia? Who gets it? What are the flow cytometry markers?

A

CLL - always older adults

Phenotype by flow cytometry:
CD19+,CD20+ (B-cell), CD5+ (T cell marker)
-> these are naive B cells which co-express B and T cell markers

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5
Q

What other B cell malignancy also co-expresses B and T cell markers and how do you tell it apart from CLL?

A

Mantle cell lymphoma (MCL)

CD23: + in CLL, - in MCL
FMC7-: - in CLL, + in MCL

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6
Q

What are good and poor prognostic factors to have by FISH for CLL? By flow cytometry?

A

FISH:
Good: del13 - miRNA
Bad: del17 - p53

Flow cytometry:
High or positive = bad: CD38 or Zap70

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7
Q

What is the most common cause of death in CLL? What does this do to blood counts as well?

A

Infections due to hypogammaglobulinemia - neoplastic B cells do not want to produce useful antibody

If they do, often causes autoimmune hemolytic anemia (leads to thrombocytopenia (Evan’s syndrome) and anemia)

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8
Q

What is Richter’s syndrome / transformation?

A

Transformation of SLL / CLL into an aggressive lymphoma, most commonly a diffuse large B-cell lymphoma

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9
Q

What is the sister disease of CLL? How exactly are they related?

A

SLL = small lymphocytic lymphoma - involvement of lymph nodes leading to generalized lymphadenopathy. Absolute leukocyte count <5k

CLL = bone marrow and blood presentation, absolute lymphocyte count >5k

Both features present = phenotypically the same = treat the same

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10
Q

How is CLL staged?

A

Via the Rai Staging
0 - monoclonal lymphocytosis >5k
I - >5k + any lymphadenopathy
II - >5k +/- lymphadenopathy + hepatomegaly or splenomegaly
III - >5k + Anemia (<11 gm/dL) +/- lymphadenopathy or organomegaly
IV - >5k + Thrombocytopenia (platelets <100K/ul) +/- lymphadenopathy or organomegaly

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11
Q

When do we treat CLL and what with?

A

Generally we treat in stages III & IV only (once anemia / thrombocytopenia have developed), though we may treat earlier if constitutional symptoms arose or lymphs are doubling faster than 6 months

Treatment is best with Rituximab (anti-CD20) + chemotherapy

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12
Q

What are common infections in late CLL?

A

Neutropenia can also occur due to marrow failure and treatment, as well as B cell dysfunction, so:

Pneumonia, cellulitis, herpes zoster

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13
Q

What type of AIHA does CLL cause and what will be seen on peripheral smear?

A

Warm antibody Coomb’s positive

Microspherocytes - small with no central pallor

Polychromatophilic RBCs - large, pale RBCs

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14
Q

How do the cells appear in prolymphocytic leukemia (PLL)?

A

They appear halfway in maturity between blast cells and mature lymphocytes of CLL

Immature: Large cells with large nucleolus

Mature: Clumped nuclear chromatin

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15
Q

What are the distinctive clinical features of PLL?

A

Massive splenomegaly WITHOUT lymphadenopathy (except for T-cell PLL)

High lymphocyte count

More aggressive than CLL with poorer prognosis -> treat more aggressively

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16
Q

What are the two types of PLL?

A

About 75% are B-cell PLL (CD20+, CD5-)

25% are T-cell PLL (CD2+, CD3+)

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17
Q

How do patients present with hairy cell leukemia (HCL) and why?

A

Usually an older male presenting with infection or fatigue

HCL causes pancytopenias which leave you susceptible to infection or anemia / fatigue

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18
Q

What will physical exam and CBC show with HCL? Why do these things happen?

A

Massive splenomegaly due to accumulation of hairy cells in red pulp

NO lymphadenopathy (similar to PLL)

Pancytopenia will be the rule
-> due to massive fibrosis of bone marrow

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19
Q

What are the lab staining features of hairy cell leukemia? How do they look on peripheral smear? What is the mnemonic?

A

Mature B cells with hairy cytoplasmic processes (appear fuzzy on LM with indistinct borders)

Lab features - TRAP + (tartrate resistant acid phosphatase)

Remember these hairy cells are TRAPPED in the bone marrow (causing fibrosis) and TRAPPED in the red pulp causing splenomegaly

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20
Q

What will happen on bone marrow aspiration of HCL?

A

Dry tap -> due to marrow fibrosis

-fibrosis caused by B cell overexpression of FGF

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21
Q

What is the prognosis / treatment of hairy cell leukemia?

A

Excellent response to cladribine (2-CDA) + rituximab, and splenomegaly

90% complete remission with once cycle of 2-CDA

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22
Q

What will the CBC show for Large Granular Lymphocytic Leukemia (LGLL) and what does the peripheral smear show?

A

CBC shows cytopenias (neutropenia, anemia) with lymphocytosis

Peripheral smear shows GRANULES in lymphocytes (vs CLL) -> shows they are NK or CD8 T cells

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23
Q

What disorder is associated with LGLL and what lab testing is done to show it?

A

Rheumatoid arthritis - autoimmune phenomenon

Lab testing shows T-cell markers or NK markers (CD56, CD57)

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24
Q

What is the treatment for LGLL?

A

Not routinely needed. Give G-CSF for neutropenia, and steroids, cyclophosphamide, and MTX if needed

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25
Q

How is PLL told apart from CLL?

A

PLL will be associated with massive splenomegaly while CLL is less so

PLL is CD23- and FMC7+
CLL is CD23+ and FMC7-, and CD5+ (T cell marker)

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26
Q

What important marker is co-expressed with CD19 in HCL?

A

CD103+

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27
Q

What are lymphomas and the two broad types?

A

Large group of disorders characterized by malignant transformation of lymphocytes - usually leading to lymphadenopathy, but may be in extranodal tissue

Broad categories:

  1. Hodgkin Lymphoma
  2. Non-Hodgkin lymphoma
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28
Q

What are the “B” symptoms of lymphomas and how do they relate to prognosis?

A

Patients are “B”othered by constitutional “B” symptoms. At least one of:

  1. Fever
  2. Night sweats
  3. Weight loss

Indicates poorer prognosis

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29
Q

How does the lymph node spread pattern differ between NHL and HL? Extranodal involvement?

A

BOth are painless, non-tender lymphadenopathy

HL - usually contiguous lymph nodes and localized

NHL - usually skips lymph nodes (noncontiguous) with more extranodal involvement common

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30
Q

Which type (NHL or HL) is more dependent on staging? What is its treatment?

A

HL is much more dependent on staging

-> radiation is mainstay of therapy

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31
Q

How is diagnosis of lymphoma made? What’s the golden rule?

A

Need a biopsy, and usually a tissue biopsy (excisional is best, fine needle aspiration won’t work)

  • > golden rule is always check CBC for lymphocytosis first in patients presenting with lymphadenopathy
  • > may be able to diagnose CLL/SLL without need for biopsy based on presence of lymphocytosis / peripheral smear
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32
Q

When is staging done and how is it done?

A

AFTER diagnosis is established
-> do via history, blood work (CBC, chemistry, titers), Imaging (PET/CT), bone marrow biopsy may be warranted in some
cases

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33
Q

What are stages I-IV of the Ann Arbor staging system for lymphomas?

A

I - involvement of one group of LN
II - involvement of 2 or more groups of LN on same side of diaphragm (above or below)
III - 2 or more groups of LN on BOTH above and below diaphragm
IV - involvement of any organs or tissues outside of lymphatic system (extranodal sites), including bone marrow

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34
Q

How do you determine stage IA or IB?

A

IB = worse prognosis, presence of “B” symptoms!!!

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35
Q

What is PET/CT?

A

An imaging procedure used for staging of lymphoma

PET - functional glucose utilization to visualize tumor activity

CT - anatomical enlargement

PET/CT = fused image

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36
Q

What would be the stage of lymphoma if patient had involvement of hilar lymph nodes alone and disease extends into adjacent lung tissue?

A

IE, not IV

Because E = extension into tissue directly adjacent to a lymph node

Note, if you had lung involvement but the lymph nodes involved were the mesenteric nodes for instance, it would be Stage IV

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37
Q

What are the most common extranodal sites of spread for lymphoma?

A

Liver and bone marrow (peritrabecular area, around the little bone spindles will be hypercellular)

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38
Q

What is the presentation of Adult T-cell leukemia/lymphoma?

A

Most common in Caribbean and Japan (endemic areas)

A diffuse erythematous rash due to skin infiltration, generalized lymphadenopathy with hepatosplenomegaly

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39
Q

What cells are present in peripheral smear of Adult T-cell leukemia/lymphoma? What causes it?

A

Associated with HTLV-1

Bizzare clover-leaf nuclei of T-cells with CD4+ phenotype

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40
Q

What are the X-ray and serum manifstations of ATLL? What could you confuse this with?

A

Lytic bone lesions, hypercalcemia

-> Easily confused with multiple myeloma, but note the presence of the rash

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41
Q

Because B symptoms can be present in NHL and HL, what are some more specific history / exam findings for HL?

A
  1. Intense pruritis

2. Alcohol-induced pain at site of disease

42
Q

What is the malignant cell in Hodgkin lymphoma? What do they look like? What is this disease associated with?

A

Reed-Sternberg cells

  • > They look like “owl-eyed nuclei” - large B cells with bilobed nuclei and prominent nucleoli
  • > associated with EBV
43
Q

How is the RS cell of HL identified and why? What markers are used?

A

Identified by immunohistochemistry (not flow cytometry) because majority of cells are reactive to the cancerous RS cells -> lymphocytes, plasma cells, macrophages, eosinophils

15 * 2 owl eyes = 30

Identified by CD15,CD30, they are actually CD20- despite being B cells

44
Q

What are the four types of classic HL? Which is most common?

A
  1. Nodular sclerosis - most common
  2. Lymphocyte-rich
  3. Mixed cellularity
  4. Lymphocyte depleted
45
Q

Give the salient features of each type of HL in terms of what makes them unique.

A
  1. Nodular sclerosis - most common, especially females
  2. Lymphocyte-rich - best prognosis
  3. Mixed cellularity - high eosinophils due to RS cells producing IL-5
  4. Lymphocyte depleted - seen in immunocompromised, worst prognosis (elderly and HIV positive)
46
Q

What is the treatment for HL?

A

Aggressive radiation in early stages, with ABVD regimen in all four stages

47
Q

What are the CBC findings in HL?

A

Normocytic anemia
Leukocytosis (reactive)
Lymphopenia (in advanced stage)
Platelets (increased early, decreased reactive)

Elevated ESR due to cytokine production

48
Q

What is the prognosis for HL and the late effects of treatment?

A

Prognosis is very good for cure

Unfortunately, high risk of later diagnosis of solid tumors from chemo / radiation

49
Q

What are the general treatment paradigms with NHL?

A

Early stage (I/II): Radiation, or chemotherapy 3 cycles + radiation if aggressive

Late stage (III/IV): Waiting, Rituximab, or chemotherapy 6 cycles +/- radiation if aggressive

Chemotherapy is R-CHOP (vs AVBD with HL)

50
Q

What marker tells you generally how aggressive a lymphoma is?

A

Ki-67 - % of cells expressing this = % of cells in S phase

~30% = low
~50% = intermediate
~80% = high aggression
51
Q

What are the indolent (low grade) lymphomas?

A

Follicular lymphoma
Small lymphocytic lymphoma (sister of CLL)
Marginal zone lymphoma (MZL)
Waldenstrom’s macroglobulinemia

52
Q

Who tends to get indolent lymphomas and what is the treatment goal?

A

Older adults

  • > will be advanced stage at presentation due to slow course
  • > often painless lymphadenopathy
  • > general intent is palliation
53
Q

What is the most common indolent lymphoma and what causes it?

A

Follicular lymphoma

  • > caused by t(14;18) -> BCL-2 gene on chromosome 18 is translocated to heavy chain locus on chromosome 14
  • > indolent because Bcl-2 inhibits apoptosis and cells slowly proliferate
54
Q

How is follicular lymphoma differentiated from follicular hyperplasia (reactive)?

A

No tingible body macrophages due to slow growth, Bcl-2 will be expressed, cells will be monoclonal, and normal lymph node architecture will be disrupted

55
Q

What are the three stages of follicular lymphoma?

A

Grade 1 - small cleaved cells
Grade 2 - mixture
Grade 3 - Large cells (getting closer to diffuse B cell lymphoma)

56
Q

What is marginal zone lymphoma? What is the most common type?

A

Neoplastic proliferation of small B cells that expands the marginal zone (post-germinal center B cells)

Most common type is MALT (mucosal associated lymphoid tissue) lymphoma

57
Q

What conditions is marginal zone lymphoma associated with? What is the treatment of a commonly tested subtype?

A

Associated with chronic inflammatory states such as Hashimoto thyroiditis, Sjogren syndrome, H. pylori gastritis

Treatment for gastric MALToma often involves treatment of H. pylori infection

58
Q

What are examples of intermediate aggressiveness lymphomas?

A

Diffuse large B cell lymphoma (DLBCL)

Mantle cell lymphoma

59
Q

What are the germinal center markers expressed by follicular lymphoma?

A

CD10 and Bcl-6

60
Q

What is the most common NHL overall? What does it usually arise from?

A

Diffuse large B cell lymphoma - with poorly differentiated cells

Arises from follicular lymphoma progression, or Richter transformation of CLL

61
Q

What causes mantle cell lymphoma? What chromosomal abnormality is associated?

A

Proliferation of small B cells that expands the mantle zone

Associated with t(11;14) - cyclin D1 from chromosome 11 is put at heavy chain locus

-> aggressive movement through growth cycle

62
Q

What are the flow cytometry markers of importance for mantle cell lymphoma?

A

Remember this is the one to differentiate from CLL because it also expresses T cell markers

CD19+, CD5+, CD23-, FMC7+

63
Q

What causes Burkitt lymphoma and what is it associated with?

A

Neoplastic proliferation of intermediate-sized B cell, associated with EBV infection

t(8;14) - movement of c-myc to heavy chain locus

Very high aggression

64
Q

What are the two forms of Burkitt lymphoma?

A
  1. Endemic form - Africa = jaw presentation in children

2. Sporadic - more loosely associated with EBV, lesion in pelvis (especially kidneys) or abdomen

65
Q

How does Burkitt lymphoma appear under the microscope?

A

Starry sky appearance from high mitotic rate background with stars of tingible body macrophages eating necrotic cells

66
Q

You find someone with cutaneous plaques made of CD4+ T cells. They are very itchy and look like psoriasis, and you find out it’s a lymphoma, but you are not in the Caribbean or Japan. What are these skin patches called?

A

Pautrier microabscesses

67
Q

What is the name of the disease causing Pautrier microabscesses, and what do the cells inside look like?

A

Mycosis fungoides

  • > cells are called Sezary cells
  • > CD4+ T cells with folded, cerebriform nucleoli
68
Q

What is Sezary syndrome? Prognosis?

A

Progression of mycosis fungoides to T cell leukemia -> sezary cells in blood.

Prognosis = indolent but incurable course

69
Q

What T-cell lymphoma is not caused by HTLV-1 and causes a nodular rash rather than psoriasis-like? How do the cells look?

A

Anaplastic Large Cell Lymphoma (ALCL)

-> cells look anaplastic and bizarre, but prognosis is actually good

70
Q

What mutation is associated with ALCL, and what markers do the cells express?

A

t(2;5)

Cells express CD30 (like R-S) cells, but do NOT express CD15

71
Q

Which Rai stages of CLL are low, medium, and high risk?

A

Low - 0 and I
Intermediate - II
High - III&IV

72
Q

Can you treat Burkitt lymphoma?

A

Yes - it is highly aggressive but a curable disease with chemotherapy

73
Q

What is Serum Protein Electrophoresis (SPEP)? How do you tell if something is monoclonal or polyclonal?

A

A way to analyze proteins in the serum

Polyclonal gamma globin peaks will be broad

Monoclonal peaks will be narrow-based (usually about the width of the albumin peak)

74
Q

What are immunoproliferative diseases and what are the usually associated with on SPEP?

A

Malignant proliferations of Ig-secreting plasma cells

Usually associated with paraproteinemia -> monoclonal band on SPEP which reflects synthesis of Ig from a single clone

75
Q

What are the classic symptoms of symptomatic myeloma? Give why all these symptoms happen

A

CRAB
Calcemia - hypercalcemia due to plasma cells activating RANK on osteoclasts, increasing bone resorption

Renal impairment - Free light chain excreted in urine damages kidney tubules, renal failure

Anemia - normo-cytic or macrocytic - Rouleaux formation

Bone disease - lytic lesions due to increased bone resportion

76
Q

What is smouldering myeloma vs symptomatic myeloma?

A

Symptomatic:

  • Monoclonal protein in serum or urine
  • > 10% plasma cells in bone marrow
  • CRAB symptoms

Smouldering:
Asymptomatic - still >10% plasma cells and monoclonal protein, but no CRAB

77
Q

What is MGUS? Why is it relevant?

A

Monoclonal gammopathy of undetermined significance
-> M spike - monoclonal spike on SPEP, but plasma cells are <10% on bone marrow and no CRAB symptoms

  • MGUS is common in older patients, and progresses to multiple myeloma at a rate of 1% per year (precursor to MM)
78
Q

What is the definition of multiple myeloma?

A

Most common primary malignancy of bone - expansion of malignant plasma cells in bone marrow, characterized by lytic bone lesions, manifestations of marrow failure, and consequences of secreted proteins

79
Q

How do plasma cells appear in MM? What markers do they express?

A

Deep blue cytoplasm with perinuclear halo where the Golgi is

Plasma cells nuclei are eccentric with clock-face chromatin

Express CD38 and CD138

80
Q

Why does bone marrow failure happen in MM?

A

Myeloma (plasma) cells are post-germinal center B cells which hone in on the bone marrow and crowd out normal hematopoiesis as they expand

-> anemia, neutropenia, thrombocytopenia

81
Q

What happens to the ESR in multiple myeloma and why?

A

It increases, because increased immunoglobulins affect the electrical surface charge of RBCs and cause them to form Rouleaux formations. Rouleaux formation settle faster in the test tube.

82
Q

What is the most common cause of death in multiple myeloma?

A

Infection -> decrease in normal serum immunoglobulins because they are suppressed by malignant clone

83
Q

What causes renal injury in MM?

A
  1. Bence Jones protein - kappa or lamda light chains deposit in renal tubules
  2. Amyloid deposition - in glomeruli and blood vessels
  3. Calcium deposition - nephrocalcinosis due to hypercalcemia
  4. Pyelonephritis - from inflammatory cells
  5. Uric acid crystal deposition (hyperuricemia from cell turnover)
84
Q

What imaging test must be avoided in patients with multiple myeloma?

A

IV contrast dye - can precipitate renal failure in patients already with renal problems

85
Q

What is the most common presenting symptom of MM? Why? How does this relate to standard workup?

A

Backache - due to bone pain -> loss of bone from osteoclast activation leads to punched out lytic lesions, especially affecting weight bearing bones

  • > compression of vertebral bodies and weight bearing bones leads to fractures
  • > Standard workup is an X-ray of all bones
86
Q

What types of antibody are normally produced in multiple myeloma?

A

IgG - 60% of cases
IgA - 20-25% of cases
Others produce only light chain

Rest are rare

87
Q

What test has replaced Bence-Jones protein detection in urine for multiple myeloma?

A

Serum free light chain (FLC)

Normal ratio is 0.6 kappa:lamda. Will be very skewed in multiple myeloma

88
Q

What protein is produced by plasma cells and has prognostic value in MM? Give the staging scheme.

A

B2-microglobulin - a part of MHC1

<3.5 mg/L is favorable (Stage I), along with albumin >3.5 g/dL
Middle: Stage II
>5.5 mg/L is poor (Stage III)

89
Q

What is the best new treatment for multiple myeloma, and what is the most common symptomatic treatment? What can you do if MM is located only at one site?

A

Only at one site - radiation may be effective

Common symptomatic treatment - bisphosphonates (for hypercalcemia and bone disease, stops osteoclasts)

Best new - Anti-CD38 (plasma cell marker) called Daratumumab

90
Q

What treatment is initially curative but often results in relapse of MM?

A

Autologous bone marrow transplant

91
Q

What condition sometimes follows multiple myeloma but is rarely a primary condition? What is the clinical definition?

A

Plasma Cell Leukemia (PCL)
>20% plasma cells in blood, with absolute plasma cell count >2k

Clinical features of pancytopenia with CRAB

92
Q

What are clinical features of amyloidosis?

A
  1. Macroglossia
  2. Cardiomegaly, CHF, arrhythmia - pressure atrophy and conduction disturbances
  3. Peripheral neuropathy
  4. Renal failure
  5. Carpal tunnel syndrome (from beta-2 accumulation)
93
Q

What is the definition of Waldenstrom macroglobulinemia?

A

Malignant proliferation of IgM-secreting plasmacytoid B lymphocytes in bone marrow
-> M spike = IgM accumulations

94
Q

How does Waldenstrom macroglobulinemia differ from MM?

A

It is associated with soft tissue disease rather than bone disease

  • > generalized lymphadenopathy, no lytic bone lesions
  • > defects are due to high IgM levels and tissue invasion of neoplastic cells
95
Q

What are the symptoms of Waldenstrom macroglobulinemia?

A

-> hepatosplenomegaly
-> cytopenias
-> generalized lymphadenopathy
Hyperviscosity syndrome w/polyneuropathy

96
Q

What is hyperviscosity syndrome and is it related to MM and/or Waldenstrom?

A

Increased blood viscosity from increased blood components (could be RBCs / WBCs)

Associated with Waldenstrom much more than MM because IgM is much larger than IgG or IgA

97
Q

What are the clinical features of hyperviscosity syndrome?

A

All due to engorgement of small vessels with impaired platelet aggregation

Visual disturbances and retinal changes due to retinal hemorrhages / occlusion

Lethargy, confusion, weakness - stroke symptoms due to poor blood flow

Epistaxis - bleeding due to small vessels in nose and poor platelet function

CHF due to increased afterload

98
Q

How is hyperviscosity syndrome treated acutely and definitively if due to Waldenstrom?

A

If due to Waldenstrom: Plasmapheresis - remove IgM

Definitively: treat underlying disease

99
Q

How do cells appear in peripheral smear in Waldenstrom?

A

Lymphoplasmacytoid -

Features in between small lymphocytes and plasma cells

100
Q

What is the mnemonic for translocations in Burkitt lymphoma, follicular lymphoma, and mantle cell lymphoma?

A

Baby, Mother, Father
8, 11, 18 (ascending size)

t(8;14) = Burkitt
t(11;14) = Mantle cell
t(14;18) = Follicular

Pathophysiology - Hematology (14 decks)

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