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Robbins Pathology Heme/Lymph (Chapters 13,14) > Chapter 13-WBC, LNs, Spleen, And Thymus > Flashcards

Chapter 13-WBC, LNs, Spleen, And Thymus Flashcards

1
Q

What is the clinical presentation of lymphocyte rich variant of Hodgkin lymphoma

A

Uncommon

  • M>F
  • Older adults
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2
Q

What are the characteristics of myelodysplastic syndromes that are associated with worse outcomes

A
  • Higher blast counts
  • more sever cytopenias
  • multiple clonal chromosomal abnormalities
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3
Q

What are some clinical presentations seen with primary myelofibrosis

A
  • Fatigue, splenomegaly, night sweats

- hyperuricemia and gout

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4
Q

What is Fanconi anemia and what are they at an increased risk for

A

Familial decreased production of all blood cells. Higher risk of acute anemia

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5
Q

What are the genetic abnormalities seen in Mantle cell lymphoma

A
  • (11;14) translocation,
  • IgH on 14
  • cyclin D1 on 11
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6
Q

What is the prognosis for AML with mutation in t(8;21)-RUNX1

A

Favorable

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7
Q

What is the genetic abnormality in large granular lymphocytic leukemia

A

STAT3

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8
Q

Which immunomarkers are present in the case of langerhans cell histiocytosis

A
  • CCR6 and CCR7 (CCR7 is only present in the neoplastic forms)
  • Binds to CCL19,20,21
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9
Q

What is the most common plasma cell neoplasm and how does it commonly present

A

Multiple myeloma, presenting as bony destruction resulting in pain due to pathological fractures

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10
Q

Which cells are the result from a hematopoietic stem cells

A

All blood cells, including the immune cells, RBCs, WBCs, platelets etc

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11
Q

What are the clinical features of large granular lymphocytic leukemia

A

Dominated neutropenia and anemia

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12
Q

Those patients with monoclonal gammopathy of uncertain significance (MGUS) are most likely to develop which condition

A

Symptomatic plasma cell neoplasm, usually multiple myeloma

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13
Q

What is the prognosis of specified peripheral T cell lymphoma

A

Worse prognosis than even the most agressive B cell lymphomas

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14
Q

Myeloid leukemias (cells with myeloid origin) originate in which locations and where do they tend to spread

A

Bone marrow that secondarily involve the spleen lymph nodes

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15
Q

What are Auer rods

A

Needle like azuroohilic granules

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16
Q

What are the common causes of neutrophilic leukocytosis

A
  • Acute Bacterial infections (especially the pyogenic conditions)
  • Sterile inflammation (such as tissue necrosis)
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17
Q

Acute lymphadenitis of the mesenteric LN region is due to drainage of which area

A

Acute appendicitis

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18
Q

What are the mechanisms that can cause the NFKB activation in Hodgkin’s lymphoma

A

EBV1 infection causing activation via:

  • activation from latent membrane protein 1 (LMP1)
  • loss of function mutation in IKB or A20 (aka TNF alpha-induced protein 3 or TNFAIP3)
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19
Q

What are the clinical features of diffuse large B cell lymphoma

A

Rapidly growing mass at the nodal or Extranodal site, typically in the Waldeyet ring, which is the oralpharnyx that includes the tonisils and adenoids

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20
Q

Splenic insufficiency causes increased susceptibility to which organisms

A

Pneumococci, meningococcal, Haemophilus influenza

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21
Q

From the third month of embryogenesis until shortly before birth, what is the location of the chief site of blood cell formation

A

HSCs migrate to the liver and become the chief site

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22
Q

Which forms of AML with t(8;21) and or inv(16) have a better prognosis

A

KIT negative mutations respond well to chemo

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23
Q

What is the cell of origin for multiple myeloma

A

Plasma B cell

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24
Q

What is the relation between smoking and leukemias

A

1.3 to 2 times higher risk of getting acute myeloid leukemia

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25
Q

What is the significance of plasma cell tumors and amyloid deposition

A

Light chains can be produced at such a rate that lambda light chains are deposited as amyloid

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26
Q

During leukocytosis, what other changes are commonly seen

A

Morphological changes:

  • toxic granulations
  • Dohle bodies
  • cytoplasmic vacuoles
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27
Q

What condition do Myeloproliferative disorders have a high propensity to develop into

A

AML

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28
Q

What are the immunomarkers in unspecified peripheral T cell lymphoma

A
  • CD2, CD3, CD5

- Alpha/beta or gamma/delta TCR

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29
Q

What is the percentage of Jones Bence proteins in multiple myeloma

A

-99%

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30
Q

What is the most common trigger for hemophagocytic lymphohistiocytosis (HLH)

A

Infection, particularly EBV

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31
Q

What are the immunomarkers in Mycosis fungoides/Sezary syndrome

A

CLA (common leukocyte antigen)
-CCR4, CCR10

*All help with the homing of T cells to the skin

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32
Q

What is the clinical presentation of follicular lymphoma

A
  • Painless, generalized lymphadenopathy

- Extranodal site involvement such as testis, GI are rare

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33
Q

What is the clinical presentation of lymphocyte depleted variant of Hodgkin lymphoma

A

-Older adult males
-HIV infected
-Advanced disease
Weightloss, fever, night sweats

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34
Q

Lymphoid neoplasms are commonly associated with what

A

-Immune abnormalities with the loss of protective immunity and the breakdown of central tolerance

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35
Q

B-All appear in which patient population most often

A

3 year old Hispanic

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36
Q

What is the most common and most deadly neoplasm of plasma cells

A

-Multiple myeloma

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37
Q

Which stain is used to see iron ladened macrophages

A

Prussian blue ( can be used to see myelodysplastic syndromes)

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38
Q

Which parasite can cause cirrhosis of the liver and subsequent splenomegaly

A

-Shistosomiasis

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39
Q

What are the clinical features of acute myeloid leukemia

A

Replacement of marrow with blasts that cause marrow failure and:

  • anemia
  • Thrombocytopenia
  • neutropenia
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40
Q

What is the classical definition of Myeloproliferative disorders

A

Increased production of one or more types of blood cells

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41
Q

What form of Reed stein burg cells are present in Nodular sclerosis variant of Hodgkin lymphoma

A

Lacunar RS cells

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42
Q

What is the state of cell infiltrate in Hodgkin’s lymphoma

A

Reactive cells as a result of the release of cytokines that are released from the Reed-Steinberg cells
-Reactive cells then further activate the RS cells via (CD30 and CD40)

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43
Q

How do multipotnent progenitors differ from HSCs

A

They are more readily able to proliferate, but have less capacity to self renew

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44
Q

What is the disease pattern seen in chronic Myeloproliferative leukemia (CML)

A
  • Slow progressing, then enters an accelerated phase, which has increased anemia and thrombocytopenia
  • The progressing into acute leukemia (aka blast crisis)
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45
Q

What is the rate that myelodysplastic syndrome (MDS) can transform into acute myeloid leukemia (AML)

A

10-40%

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46
Q

What is the prognosis for AML with mutation in NPM

A

Good

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47
Q

What is the immunomarkers in the case of the classic variants of Hodgkin lymphoma

A

Positive for PAX5, CD15,CD30

Negative for CD 19, 20, 45

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48
Q

What is the morphological appearance of Dohle bodies and when are the commonly seen

A

Patches of dilated ER that appear as sky-blue cytoplasmic “puddles” seen during leukocytosis

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49
Q

Thrombosis of the splenic vein cause be caused by and can raise suspect of which conditions

A

-Carcinomas of the stomach or pancreas

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50
Q

What are the characteristics of the LNs in acute lymphadenitis

A

Enlarged and painful

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51
Q

What is the chromosome finding commonly seen in reed steinberg cells

A

Gain of function in the REL proto-oncogene on chromosome 2p

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52
Q

What are the contents of a thymoma

A

-rumors of thymic epithelial cells, contains benign immature T cells

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53
Q

What is a major complication of the BCR-ABL inhibitors

A

Resistance to it, which occurs in 50% of patients

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54
Q

Where do most Myeloproliferative disorders originate from

A

Multipotent myeloid progenitors

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55
Q

What are the clinical presentations of someone with splenomegaly

A
  • dragging sensation in the LUQ, with pressure in the stomach,especially after eating
  • Anemia
  • leukopenia
  • thrombocytopenia
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56
Q

What are the immnofactors involved in Burkitt’s lymphoma

A

CD10, CD19, CD20, BCL6

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57
Q

What is ataxia telangiectasias and what are they at an increased risk for

A

Caused by autosomal recessive of ATM gene

-Neurodegenerative Disease that also carries an increased risk for acute leukemia

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58
Q

What are the markers that can be seen in the blood as the result of the tumor of a plasma cell

A
  • M component aka, the Ig marker of the tumor

- can be complete Ig, but commonly have excess light chains

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59
Q

What is the function of the MYC abnormality that allows for tumor development

A

Allows the Warburg effect, which allows shunting for aerobic glycolysis, allowing availability of glucose and glutamine

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60
Q

Which form of the diffuse large B cell lymphoma has a poorer prognosis

A

MYC translations

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61
Q

The proliferation and survival of multiple myeloma cells are dependent on which factor

A

IL6

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62
Q

What are the characteristics of the spent phase of polycythemia Vera (PCV)

A

Extensive fibrosis of the marrow that displaces the hematopoietic cells, usually resulting in extrameduallry hematopoiesis and organomegaly

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63
Q

What is the prognosis of mantle cell lymphoma

A

Poor, 3 to 4 years survival

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64
Q

What is the most common form of indolent NHL

A

Follicular lymphoma

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65
Q

Multifocal unisystem langerhans cell histiocytosis primarily affects which organs/factors

A
  • Bone lesions that expand into the adjacent soft tissue

* Develops diabetes insipidus in 50% of pts due to involvment of the posterior stalk of the hypothalamus

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66
Q

Chronic lymphocytic leukemia (CLL) signals are transduced through which pathway

A

Breton tyrosine kinase (BTK)

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67
Q

What is the clinical presentation of mantle cell lymphoma

A
  • Painless lymphadenopathy
  • involvement of the GI or spleen
  • polyp like lesions in the gut or colon (lymphomatoid polyposis)
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68
Q

What are the histological findings in primary myelofibrosis

A
  • atypical megakaryocytes (cloud like)

- teardroped shaped red cells (dacryocytes)

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69
Q

Which AML has high number of Auer rods

A

T(15;17) aka acute promyelocytic leukemia

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70
Q

Which cell type tends to be increased in essential thrombocytosis (ET)

A

Megakaryocytes, usually with enlarged platlets

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71
Q

What are the histological findings in adult T cell lymphoma/leukemia

A

Multilobular nuclei in CD4 cells, usually in the shape of “flower or cloverleaf” shape

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72
Q

In AML that develops de novo in younger adults will have which translocations

A

Balance translocations of:

  • t(8;21)
  • t(15;17)
  • inv(16)
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73
Q

What are the genetic abnormalities seen in essential thrombocytosis

A
  • JAK2 (50% of cases)
  • MPL (5-10%)

*most have mutations in calreticulin

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74
Q

What is the association between EBV and the modular sclerosing variant of Hodgkin lymphoma

A

None

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75
Q

What is leukocytosis

A

Increased number of white blood cells in the blood

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76
Q

In addition to adult T cell lymphoma/leukemia, what condition are patients with HTLV-1 at risk for

A

-progressive Demyelinating disease of the CNS and spinal cord

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77
Q

A patient with B-ALL will present with which clinical features

A
  • lymphadenopathy
  • Splenomegaly
  • Bone pain
  • hepatomegaly
  • testicular enlargement
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78
Q

What are the complications that can be seen and fatal to a patient with primary myelofibrosis

A
  • intercurrent infections
  • thrombotic episodes
  • bleeding with platlet abnormalities
  • transformation to AML (5-20%)
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79
Q

Which cells are considered to be colony forming units

A

All cells from the myeloid lineage, which are all but NK, T and B cells

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80
Q

What are the most common genetic abnormalities in chronic lymphocytic leukemia (CLL)

A 
*Translocations are rarem but
deletions of:
-13q14.3
-11q
-17p
Trisomy of 12q
Gain of fucntion in NOTCH 1
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81
Q

What are the immunofactors that are found on the cells in chronic lymphocytic leukemia (CLL)/Small lymphocytic lymphoma (SLL)

A

Pan B cell markers, which are CD, 5, CD19, CD20, CD23

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82
Q

What are the clinical features of unspecified T cell lymphoma

A

-Lymphadenopathy with eosinophilic, pruritis, fever, weight loss

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83
Q

Which virus is implicated in the production of a malignancy that presents as a malignant infusion in the pleural cavity

A

HHV8

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84
Q

What are the factors that can cause the leukocytosis via the mechanism of decreased extravasation in tissues

A

-Glucocorticoids

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85
Q

What is the cell of origin in Mycosis fungoides/Sezary syndrome

A

CD4 cells that hone to the skin

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86
Q

Which more serious condition can PCV develop into

A
  • ALL is very rare

- AML in 2%

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87
Q

What is the prognosis of diffuse large B cell lymphoma

A

Aggressive tumors that are fatal without treatment:

-intensive chemo has a 60-80% of complete remission with 40-50% cured

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88
Q

Which form of multiple myeloma is associated with a poorer prognosis

A

TP53

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89
Q

Which mutations in small amounts of MDS that are associated with a bad prognosis

A

TP53

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90
Q

IN normal adults, what is the ration of fat cells to the hematopoietic elements, and how does this ration change in disease states

A

Normal 1:1
Hypoplasia states: more fat cells
Tumors: less fat cells

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91
Q

What are the clinical causes of death in multiple myeloma

A
  • Recurrent bacterial infections

- Renal insufficiency

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92
Q

Which blasts ahve a delicate nuclear chromatin, two to four nucleoli, and more voluminous cytoplasm

A

Myeloblasts

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93
Q

Which conditions increase the risk for a splenic rupture

A
  • infectious mononucleosis
  • malaria
  • typhoid fever
  • lymphoid neoplasms

*due to the rapid increased size of spleen resulting in thinning of the capsule

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94
Q

What is the characteristic feature of follicular lymphomas

A

Devoid of any apoptotic B cells (due to the hover expression of the survival molecule BCL2

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95
Q

Where do marginal zone lymphomas arise from

A

LNs, spleen, or Extranodal tissues, usually in the case of chronic inflammation

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96
Q

What are the immunomarkers in the case of nonclassic Hodgkin lymphoma

A

Positive for CD19,20, BCL6

Negative for CD15, 30,

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97
Q

What is the clinical presentation of nodular slcerosing variant of Hodgkin lymphoma

A
  • stage 1 or 2 disease
  • Mediastinal involvement
  • most patients young adults, M=F
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98
Q

What is the common genetic even in all of Hodgkin’s lymphoma

A

Activation of the NFKB pathway

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99
Q

What is the characteristics of the lymphadenopathy seen in Hodgkin lymphoma

A

Painless

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100
Q

What is the peak age for acute myeloid leukemia (AML)

A

60 years old

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101
Q

What is the prognosis for AML with mutation in t(15;17)-RARA

A

Intermediate

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102
Q

What is the significance of increased number of light chains in the process of the plasma cell tumors

A

-Light chains, known as Bence-Jones proteins, are excreted in the urine

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103
Q

What is the most common plasma cell, especially occurring in older patients

A

Monoclonal gammopathy of uncertain significance (MGUS)

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104
Q

Most common AML in patients with Down syndrome and which morphology findings are common

A

AML with megakaryocytes maturation with detection of Abs against GIIb/IIa or vWF (megakaryocytes specific markers), commonly have marrow fibrosis

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105
Q

Which factors are upregulated in the cause of marginal zone lymphomas

A

BCL10 and MALT1

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106
Q

What is meant by the term pluripotency

A

Ability to become any of the mature cells in the blood

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107
Q

What is the clinical presentation of multiple myeloma

A
  • Lytic bone lesions
  • Hypercalcemia
  • Renal failure
  • immune abnormalities
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108
Q

What are the organisms patients with agranulocytosis are at a high risk of contracting

A
  • Candida

- Aspergillus

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109
Q

When do blood cell progenitors first appear and from which location

A

Yolk sac in the third week of gestation development

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110
Q

What is the true about anaplastic large cell lymphoma to have a poor prognosis

A

Lack of ALK gene, seen in older populations

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111
Q

What is the state of the capsule in a thymoma

A

Capsules are present, although can be penetrated in 25% of the time

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112
Q

Which genetic abnormality in follicular lymphoma is commonly seen

A

t(14;18), leading to overexpression of BCL2

  • 14=IgH location
  • 18= BCL2 location
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113
Q

What is the organ order of disease in Hodgkin lymphoma

A

1) Nodal Disease
2) Splenic disease
3) hepatic disease
4) marrow and other tissues

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114
Q

What is the condition of leukoerythroblasosis

A

Distortion of the marrow architecture that cause the immature release of precursors in to the blood

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115
Q

Which characteristic of chronic lymphocytic leukemia will follow a more aggressive course

A

-unmutated Ig segments

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116
Q

Which genetic abnormalities are associated with a better prognosis in multiple myeloma

A

-cyclin D1

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117
Q

What are the histological findings in the cause of Burkitt’s lymphoma

A
  • High mitotic index, with numerous apoptotic cells

- High rate of apoptotic cells leads to macrophage engulfment and the “starry sky” appearance

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118
Q

What is the time frame that drug induced MDS, myelodysplastic syndrome arise (also known as t-MDS)

A

2 to 8 years later

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119
Q

When might a marginal zone lymphoma regress

A

Upon removal of the pathogen or inflammation, as in the case of antibiotics to treat H pylori

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120
Q

Which form of AML as the best prognosis and what is the reasoning

A

T(11;15) because of treatment with all trans retinoic acid and arsenic salts

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121
Q

Adult T cell lymphoma/leukemia has what genetic abnormalities

A

Tax, which activates NFKB

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122
Q

What is the progression of disease in Mycosis fungoides/Sezary syndrome

A

1) Premycotic phase
2) plaque phase
3) tumor phase

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123
Q

Which from of myelodysplatic syndromes progresses to AML at the highest frequency

A

t-MDS, as drug induced MDS

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124
Q

What are the features of a reactive hyperplasia that is non neoplastic

A

1) Preservation of the LN architecture
2) Marked variation of the shape and size of the follicles
3) frequent mitotic figures, phagocytic macrophages with recognizable light and dark zones

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125
Q

What is the prognosis of Mycosis fungoides/Sezary syndrome and what is the usually the fatal event

A

Indolent tumors, with the progression to Agressive T cell lymphoma being the terminal event

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126
Q

What is the amino acid substitution in the case of polycythemia Vera (PCV)

A

Valine to phenylalanine at 617

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127
Q

What is the association between EBV and the lymphocytic depleted variant of Hodgkin lymphoma

A

90%

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128
Q

What is the general prognosis in the cause of Hodgkin’s lymphoma

A

Good

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129
Q

What is the prognosis of chronic lymphocytic leukemia (CLL)

A

Depends on the clinical stage of the disease

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130
Q

AMLs in older adults tend to have which genetic abnormalities as what is the prognosis

A

-deletions of 5q or 7q and are bad prognosis

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131
Q

What is the most common cancer of children

A

Acute lymphoblastic leukemias/lymphomas (ALLs)

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132
Q

What are the clinical features of an Extranodal NK/T cell lymphoma

A
  • Destructive nasopharyngeal mass, with less commonly in testis and skin
  • Causes ischemic necrosis
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133
Q

Which patient population is hairy cell leukemia seen

A

Middle aged white males (5:1)

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134
Q

In those patients that received alkylating agents for Hodgkin lymphoma were at higher risk to develop what conditions

A

Acute myeloid leukemia

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135
Q

What are the histological features of classic Reed-steinburg cells

A

-Large cells with multiple nuclei or single nucleus with multiple nuclear lobes. Each with a large inclusion like nucleolus about the size of a small lymphocytes

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136
Q

Univocal unsystematic Langerhans cell histiocytosis primarily affects which location

A

Skeletal system

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137
Q

What are tertiary lymphoid organs

A

Chronic immune reactions that cause the appearance of organized collections of immune cells in non lymphoid tissue

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138
Q

What are the common causes of eosinophilic leukocytosis

A
  • Allergic disorders (asthma, hay fever, etc)
  • Parasitic
  • autoimmune
  • Atheroembolic disease
139
Q

What form of Reed stein burg cells are present in mixed cellularity variant of Hodgkin lymphoma

A

Mononuclear RS cells

140
Q

Which cells are nonspecific esterase positive

A

Monoblasts

141
Q

What is the most common cause of agranulocytosis

A

Drug toxicity

142
Q

What are the two most common translocations in the cause of AML

A

1) t(8;22)- RUNX1
2) inv(16)- CBFB

*blocking the maturation of myeloid development

143
Q

What is the prognosis of Burkitt’s lymphoma

A

Very agressive but responds well to treatment

144
Q

What is the diagnosis in a patient that has LNs with:
-infiltrate of small lymphocytes with round to irregular nuclei, condensed chromatin, and scant cytoplasm with admired lymphocytes that batter in loose aggregates

A

Chronic lymphocytic leukemia (CLL)/Small lymphocytic lymphoma (SLL)

*What is described is a proliferation center, and is pathogonomic

145
Q

What are the surface markers of hairy cell leukemia

A
  • CD19,CD20

- CD11c, CD25, CD103, annexin A1

146
Q

What is the most common aneuploidy seen in myeloid cancers

A

MYC on chromosome 8

RPS14 on chromosome 5q

147
Q

What are patients with polycythemia Vera (PCV) at highest risk for

A

Hyperviscosity of blood
Thrombocytosis
Abnormal platlets
*all leading to thrombosis and bleeding

148
Q

In Hodgkin’s lymphoma, what is the cell of origin

A

Germinal B cells

149
Q

What is the hallmark of primary myelofibrosis

A

Development of obliterative marrow fibrosis

150
Q

What is the causes of congestive splenomegaly

A

Chronic venous outflow obstruction, usually seen due to:

  • Cirrhosis of the liver
  • Portal or splenic vein thrombosis
  • cardiac failure

*all lead to portal or splenic hypertension

151
Q

What is the meaning of leukopenia

A

Deficiency of leukocytes

152
Q

What is the lymphocyte count that is required to diagnose chronic lymphocytic leukemia (CLL)

A

> 5000 per mm

153
Q

What is the age group that myelodysplastic syndrome mostly affects

A

Older adults around 70 years old

154
Q

Which histological stain is helpful in the distinction between lymphoblastic and myeloblast

A

Lymphoblasts are myeloperoxidase negative and contain periodic acid-Schaffer’s positive cytoplasmic material

155
Q

What are the histological features of mononuclear variant Reed-steinburg cells

A

Large cells with a single nucleus with a large inclusion like nucleolus

156
Q

What is the genetic abnormality seen in langerhans cell histiocytosis

A

-valine to glutamate substitution at the BRAF gene (commonly seen in hairy cell leukemia as well and seems to be present in this disease 55-60% of the time)

157
Q

Mantle cell lymphoma are of which cell type

A

Mantle zone B cells

158
Q

What are the factors that indicate a good prognosis with ALL

A
  • 2-10 years of age
  • low white count
  • hyperdiploidy
  • trisomy of 4,7,10,
  • t(12;21)
  • t(9;22)
159
Q

T-ALLs have which genetic mutation

A

gain of function in NOTCH1 (70%)

160
Q

What are the two cell types seen morphologically in follicular lymphoma and which is more common

A

1) Small cells with irregular or cleaved nuclear contours and scant cytoplasms known as centrocytes*** more common
2) Large cells with open nuclear chromatin, several nuclei, modest cytoplasm known as centroblasts

161
Q

How does an acute viral infection result in lymphopenia

A

The production of type 1 interferons causes the sequestration of T cells into the LNs and increased adherence to vessel walls as a result of changes the adhesion molecules on the T cells

162
Q

What are the clinical features of chronic lymphocytic leukemia (CLL)

A

Usually asymptomatic, but when they do present

  • Lymphadenopathy, hepatosplenomegaly
  • hypogammaglobulinemia
  • Increased risk for infection
163
Q

Chromosome 13 deletions in chronic lymphocytic leukemia (CLL) are indicated with changes in which factors

A

Micro-RNAs of miR-15a, miR-16-1

164
Q

What is the characteristic histological feature in the cause of Langerhans histiocytosis

A

Birbeck granules, which are pentaluminal tubules, which often have a dilated end that looks like a tennis racket (contains langerin)

165
Q

During the blast crisis of chronic Myeloproliferative leukemia (CML), what is the genetic abnormality

A

Mutations that mess up Ikaros, the transcription factors, similar to the B-ALL leukemias

166
Q

Where do the majority of megakaryocytes reside and what do they produce

A

Resides next to sinusoids in bone marrow,and produce platelets

167
Q

What are the clinal features of essential thrombocytosis

A

Elevated platlets, but no polycythemia or myelofibrosis

168
Q

What are the immunofactors in multiple myeloma

A
  • CD138, aka an Adhesion molecule known as syndicate-1

- CD56

169
Q

Polycythemia Vera (PCV) shows which clinical features

A

Increased marrow production of:

  • RBCs
  • Granulocytes
  • Platlets
170
Q

Which patients are seen to have chronic lymphocytic leukemia (CLL)

A

60 year old male

171
Q

The genetic abnormality in anaplastic T large lymphoma results in what

A

ALK gene causes constitutively activated tyrosine kinase

172
Q

How do 2/3 of non-Hodgkin lymphomas all of Hodgkin lymphomas present

A

Enlarged, nontender LNs

173
Q

All cases of lymphoplasmacytic lymphoma involve mutations of which factor

A

MYD88

174
Q

What are patients with hairy cell leukemia at a higher rate for

A

Infection, especially atypical mycobacterium

175
Q

What is a serious side effect of the viscosity of the blood seen in PCV patients

A
  • Plethoras and cyanosis due to the stagnation and deoxygenation of blood in the peripheral vessels
  • Budd chiari syndrome (thrombosis of hepatic vein), portal vein and mesenteric vein, all leading to potential bowel ischemia
176
Q

What is the association between EBV and the mixed cellularity variant of Hodgkin lymphoma

A

70%

177
Q

What is the clinical presentation of lymphocyte predominant variant of Hodgkin lymphoma

A

Young males with cervical or axillary lymphadenopathy, mediastinal

178
Q

What is the triad of felty syndrome and what condition is commonly seen to be the underlying cause

A

Triad:
RA, splenomegaly, neutropenia
-Usually have large granular lymphocytic leukemia as the underlining cause

179
Q

What are the factors that can cause the leukocytosis via the mechanism of increased release from the marrow stores

A
  • Endotoxemia
  • infection
  • Hypoxia
180
Q

AML arising following treatment with topoisomerase inhibitors commonly have which genetic abnormalities

A

Translocation of MLL gene on 11q23

181
Q

What are the typical protein findings in the case of multiple myeloma

A
  • Increased IgG, Kappa light chain

- Decreased lambda ligh chain levels

182
Q

Which genetic abnormalities are commonly seen in MDS and tMDS

A
  • monosomies 5 and 7
  • deletions of 5q, 7q, 20q,
  • trisomy 8
183
Q

Stem cell leukemia is due to which mutation

A

FGFR1 fusions

184
Q

Which infections are AML patients prone to

A

Pseudomomnas, fungi and commensal

185
Q

What is the diagnostic feature of the Nodular sclerosing variant of Hodgkin lymphoma

A

-Collagen depositions in bands that divide the LNs into circumscribed nodules

186
Q

What are the two variations of large granular lymphocytic leukemia and what are their immunomarkers

A

T cells variant: CD3 pos, CD56 neg

NK variant: CD56 pos, CD3 neg

187
Q

Other the LN involvment, what other locations are seen to be affected in follicular lymphoma and what pattern is seen there

A
  • Bone marrow involvement, showing paratrabecular lymphoid aggregates
  • splenic white and hepatic portal triads
188
Q

How do the B and T cell acute lymphoblastic leukemias present

A

B cells: childhood acute leukemias

T cells: adolescent male lymphomas

189
Q

What is the result of MYC translocations

A

A progrowth mutation:

  • Increased cell division
  • Warburg effect
190
Q

What are the factors that are present on the stem cells

A

CKIT+
Sac-1+
LIN-

191
Q

What is the prognosis of adult T cell lymphoma

A

Very bad, fatal in a year despite chemo

192
Q

What is the prognosis of multifocal multisystem langerhans cell histiocytosis

A

With intensive chemotherapy, 50% survive

193
Q

Late stage and highly aggressive forms of multiple myeloma are associated with which genetic abnormality

A

MYC

194
Q

What form of Reed stein burg cells are present in lymphocytic predominant variant of Hodgkin lymphoma

A

-L and H RS cells

195
Q

In addition to BCL2, follicular lymphomas commonly express which gene

A

MLL2, which is a histone methyltransferase

196
Q

What condition will have “smudge cells”, which are cells that were disrupted in the process of the smear

A

Chronic lymphocytic leukemia (CLL)/ Small lymphocytic lymphoma (SLL)

*looks like a cell exploded almost

197
Q

What are the immunomarkers in the cause of langerhans cells histiocytosis

A

HLA-DR, S100, CD1a

198
Q

What is the characteristic histiological finding in chronic Myeloproliferative leukemia

A

Presence of macrophages with abundant wrinkled, green-blue cytoplasm so called sea blue histiocytes

199
Q

What is the prognosis of Extranodal NK/T cell lymphomas

A

aggressive and depends on the stage of the disease, as it responds well to ration but not to chemo

200
Q

In those patients with hemophagocytic lymphohistiocytosis that survive, what other affects are seen

A
  • Renal damage in adults

- Growth and mental retardation in children

201
Q

What are the major complications seen in the case of essential thrombocytosis

A

-Thrombosis and hemorrhage due to dysfunctional platelets

202
Q

What is the histological finding in anaplastic large cel lymphoma

A

-hallmark “horseshoe cells” which are horseshoe nuclei with voluminous cytoplasms

203
Q

What are the factors that can cause the leukocytosis via the mechanism of increased production in the marrow

A
  • Chronic infection/inflammation
  • Paraneoplastic
  • Myeloproliferative disorders
204
Q

Which immuno markers are present in follicular lymphomas

A

-CD10, CD19, CD20, BCL6

205
Q

Acute lymoblastic leukemia/lymphoma are mainly composed of which cells

A

Premature B cells (85%) with the remainder of premature T cells

206
Q

Invasive thymomas have which characteristic

A
  • cytologically benign but locally invasive, with propensity to metastasize
  • by definition, they penetrate the capsule
207
Q

What factors correlate with a worse outcome in patients with chronic lymphocytic leukemia (CLL)

A
  • deletions of 11q and 17p
  • lack of somatic hypermutation
  • ZAP-70
  • NOTCH 1
208
Q

What is the location of disease in Mycosis fungoides/Sezary syndrome

A
  • epidermis and upper dermis in early disease

- Bone marrow and LNs

209
Q

Which patients are seen to have adult T cell leukemia/lymphoma

A

Only those with HTLV-1 (retrovirus that infects CD4 cells)

-Commonly in southern Japan, West Africa, Caribbean basin

210
Q

During the accelerated phase of chronic myeloproliferative leukemia (CML) which genetic abnormalities arise

A
  • Trisomies 8
  • Isochromosome 17q
  • duplication of the Philadelphia translocation
211
Q

What is the prognosis for AML with mutation in inv(16)-CBFB

A

Good

212
Q

What cell origin in marginal zone lymphomas

A

Memory B cells

213
Q

What are the common features of all forms of Hemophagocytic lymphohistiocytosis (HLH)

A

Reactive reaction:

1) Activation of macrophages and CD8 cytotoxic T cells
2) Proinflammatory cytokine “stew”
3) Suppression of hematopoiesis and cytopenias
4) Shock or cytokine storm

214
Q

What is the prognosis of patients with unifocal or multifocal unisystem langerhans cell histiocytosis

A

Good, as there are tendencies to self regression

215
Q

What is systemic mastiocytosis

A

The mass production of mast cells, which are located throughout the tissues

216
Q

What are the clinical presentations of AML

A

Fatigue, fever, spontaneous mucosal, cutaneous bleeding:

  • thrombocytopenia
  • anemia
  • neutropenia
217
Q

What is the prognosis of ALL

A

Very good, with 95% remission and 75-85% cured

218
Q

IN the cause of Burkitt’s lymphoma, what histological finding would indicate involvement of the bone marrow

A

-tumor cells with slightly clumped nuclear chromatin, 2-5 distinct nucleoli, royal blue cytoplasm containing clear cytoplasmic vacuoles

219
Q

Most lymphoid neoplasms are of which origin

A

B cell origin (85-90%), with T cells making up the majority of the remainder

220
Q

What is rouleaux formation and which condition is it present in

A

-High levels of M proteins in multiple myeloma causes red cells in peripheral blood smears to stick to one another in linear arrays

221
Q

Where do the majority of red blood cell precursors reside

A

Surrounds macrophages (aka nurse cells) which provide the iron for the production of hemoglobin

222
Q

B-ALLs have which genetic mutation

A
  • Loss of function in PAX5, E2A, EBF

- t(12;21), ETV6 and RUNX1

223
Q

AMLs that arise due to DNA damaging agents, commonly used to treat myelodysplastic syndrome have which genetic abnormalities

A

deletions or monosomies of:

  • 5q
  • 7q
224
Q

What is the risk factor in patients with follicular lymphoma

A

Development into diffuse Large B cell lymphoma (30-50%)

225
Q

What is the clinical presentation of Burkitt’s lymphoma

A

Extranodal sites

  • Endemic: mass in the mandible, the abdominal viscera, especially the kidneys, ovaries, adrenal gland
  • Sporadic: illeocecum and peritoneum
226
Q

What are the histological characteristics of the L and H variant of Reed Steinberg cells

A

“Popcorn cell” with multilobular nucleus

227
Q

What is the level of neutropenia that puts a patient at a high risk of developing an infection

A

-500 per mm3

228
Q

Which form of Hodgkin’s lymphoma is differently than the others

A

-Lymphocyte predominate has a abnormal Reed-Sternberg cells

229
Q

Which blasts have folded or lobulated nuclei, Lack Auer rods, and are nonspecific esterase positive

A

Monoblasts

230
Q

Which three viruses are implicated in the increased risk of lymphomas

A
  • EBV
  • human T cell leukemia virus (HTLV-1)
  • Karposi (HHV-8)
231
Q

What gene product is affected in AML with a translocation of t(11q23;v)

A

MLL

232
Q

Which form of AML tends to exacerbate the bleeding tendencies

A

T(11;15)

233
Q

What is the diagnosis of AML based on

A

Presence of at least 20% blasts in the bone marrow

234
Q

Which age group is commonly seen to develop diffuse large B cell lymphoma

A

60 year old adults

235
Q

How do patients with lymphoplasmacytic lymphoma present clinically

A
  • lymphadenopathy
  • Hepatomegaly
  • Splenomegaly
236
Q

What is the durability of CML

A

There is not one, the BCR inhibitors can help to reduce the load, but the “stem cell” is not hit, therefore incurable

237
Q

What is the characteristic of marginal zone lymphomas on its ability to spread

A

Tend to stay localized from some time, spreading only late in the course

238
Q

What is the prognosis of chronic lymphocytic leukemia where there is the development of a rapidly enlarging mass writhing the lymph node or spleen

A

Very bad (<1 year), and is due to the transformation into the large diffuse B cell lymphoma known as Richter syndrome

239
Q

What genetic abnormalities are seen in hairy cell leukemia

A

BRAF serine/threonine kinase, particular a valine to glutamate a residue 600

240
Q

In the case of polycythemia Vera (PCV) that has two mutated copies of JAK2, which clinical events are seen

A
  • Higher white cell count
  • More splenomegaly
  • symptomatic pruritis
  • Greater rate of progression to the spent phase
241
Q

IN the case of large granular lymphocytic leukemia, what does the prognosis depend on

A
  • T cells variant are more indolent

- NK variant is more aggressive

242
Q

IN the cause of diffuse Large B cell lymphoma, what is seen in pimrary effusion lymphoma and what is the cause

A

The cause is HV8 from Karposi sarcoma causing:

-Malignant pleural or ascitic effusion

243
Q

What do mutations in BCR-ABL cause

A

Proliferation of granulocytic and megakaryocytes progenitors, along with the release of immature ones from the marrow

244
Q

What is the mutation seen in primary myelofibrosis

A

JAK2 (50%)
MPL (1-5%)

*mutations in calrectulin as well)

245
Q

What increased cell is seen in during a boretella pertussis Infection

A

-Lymphocytosis

246
Q

Which genetic abnormalities are associated with a worse prognosis in multiple myeloma

A
  • deletions of 13q
  • Deletions of 17p
  • translocation of t(4;14)
247
Q

What are the presenting features of hemophagocytic lymphohistiocytosis (HLH)

A
  • Splenomegaly and hepatomegaly
  • Anemia
  • Thrombocytopenia
  • High levels of plasma ferritin
  • soluble IL-2
248
Q

What age group is affected with eh multifocal multisystem Langerhand cell histiocytosis

A

Aka Letterer-Size disease

Children

249
Q

What is the location of a tertiary organ in the cause of H. Pylori

A

-mucosal lymphcytes in the Payer’s patches

250
Q

When and where hematopoietic stem cells first appear

A

Several weeks after the appearance of the cell progenitors, arising from the mesoderm of the intraembryonic aorta, gonad, mesonephric regions

251
Q

What form of Reed stein burg cells are present in Lymohocytic depleted variant of Hodgkin lymphoma

A

Reticular/pleomorphic RS cells

252
Q

What is the cell type present in noninvasive thymomas

A

Medullary like epithelial cells or a mixture of medulla and cortical cells

253
Q

Acute lymphadenitis of the cervical region is due to drainage of which area

A

Infections of the teeth of tonsils

254
Q

What are the immunomarkers in Extranodal NK/T cell lymphoma

A
  • CD21 and CD3 neg (no TCR)

- NK Type markers (CD56 positive)

255
Q

What are the histological features of an invasive thymoma

A
  • Cortical variety
  • abundant cytoplasm
  • rounded vesicular nuclei
256
Q

How can a myeloblast be differentiated from lymphoblastic tumors

A

Lymphoblastic have:

  • more condensed chromatin
  • less clear nucleoli
  • small amounts of cytoplasms that lack granules
257
Q

What are the common causes of basophilic leukocytosis

A

-Rare, but commonly a sign on Myeloproliferative disease

258
Q

What are the histological findings in myelodysplastic syndromes

A
  • Ring sideroblasts (iron ladened macros)
  • megaloblastoid maturation
  • nuclear budding abnormalities (nuclei with misshapen and polyploid outlines
  • Neutrophils with decreased granules (aka Dohle bodies)
  • Pseudo-Pelger-Huey cells (neutrophils with two nuclear lobes
259
Q

What is the histological finding of peripheral T cell lymphoma

A

Spectrum of small, intermediate, and large lymphoid cells, usually with irregular nuclear contours

260
Q

What is the association between EBV and the lymphocytic dominant variant of Hodgkin lymphoma

A

None

261
Q

What is the immunomarker in the cause of anaplastic large cell lymphoma

A

CD30

262
Q

What is hand-Schuyler-Christian syndrome

A
  • Calvarial bone defects
  • diabetes insipidus
  • exopthalamos
263
Q

Acute lymphadenitis of the axillary region is due to drainage of which area

A

Infections of the extremities

264
Q

A patient with T-ALL will present with which clinical features

A
  • mediastinal thymic masses causing vessel compression
  • lymphadenopathy
  • Splenomegaly
  • Bone pain
  • hepatomegaly
  • testicular enlargement
265
Q

What are the immune receptors seen in diffuse large B cell lymphoma

A

CD19, CD20, Ig receptors

266
Q

What is the morphological appearance of toxic granules and when are the commonly seen

A

-Seen in leukocytosis, and appear as coarser and darker than normal granules

267
Q

In the case of multiple myeloma secreting IgA and IgG, what complications arise

A

Increased viscosity

268
Q

The familial forms of hemophagocytic lymphhistiocytosis (HLH) are associated with which central defect

A

Dysfunction in the ability of cytotoxic T cells and NK cells to form or deploy cytotoxic granules

269
Q

What is the first symptom commonly seen in chronic Myeloproliferative leukemia (CML)

A

Dragging sensation in the abdomen, commonly due to splenomegaly or acute onset of pain in the upper right quadrant due to splenic infarct

270
Q

What is the effect of splenomegaly on the neutrophil count

A

Results in neutropenia as a result of increased sequestration

271
Q

Hairy cell leukemia is of which cell origin

A

Memory B cell

272
Q

What are the most common locations for there to be an emboli lodging

A

Spleen, kidney, brain

273
Q

What is the prognosis of hemophagocytic lymphohistiocytosis

A

Grim without treatment, particularly if they have the familial form

274
Q

Which two common conditions place patients at an increased risk of childhood leukemia

A
  • Down syndrome (trisomy 21)

- Type 1 neurofibromatosis

275
Q

What is the result of result of sulfonamides on neutrophils and what is the mechanism of actions

A

-Agranulocytosis as a result of antibody mediated destructions of the mature neutrophils

276
Q

What is the clinical presentation of mixed cellularity variant of Hodgkin lymphoma

A
  • Stage 3 or 4
  • M>F
  • peaks in young adults and older than 55
  • Weightloss, night sweats, fever
277
Q

IN the cause of diffuse large B cell lymphomas, what are two pathogens that can cause it

A
  • EBV in the case of immunodefiecncy

- HHV8 (Karposi) that causes primary effusion lymphoma,

278
Q

In those patients that received radiation for Hodgkin lymphoma were at higher risk to develop what conditions

A
  • Lung cancer
  • Melanoma
  • breast cancer
279
Q

Which patient population is seen to develop mantle cell lymphoma

A

50-60 year old males

280
Q

What pathogenesis is associated with Extranodal NK/T cell lymphoma

A

EBV

281
Q

What is true about anaplastic large cell lymphoma to have a good prognosis

A

ALK positive, usually seen in children and young adults

282
Q

Where does anaplastic large cell lymphoma congregate

A

Around venules and infiltrate lymphoid sinuses, which can mimic metastatic carcinoma

283
Q

What is the dominant clinical feature in the cause of multifocal multisystem langerhand cell histiocytosis

A
  • Cutaneous lesions that resemble a seborrheic rupture, which are infiltrates of langerhans cells over the front of the back trunk and scalp
  • anemia, thrombocytopenia, recurrent infections, otitis media, mastoiditis
284
Q

How does the characteristic of hairy cell leukemia affect its ability to be aspirated

A

-Stuck in a extracellular matrix of reticulin fibers, so produces a “dry tap”

285
Q

What is the prognosis for AML with mutation in t(11q23;v)-MLL

A

Poor

286
Q

What is the cellularity in the cause of agranulocytosis in the cause of the drugs that suppress or destroy the granulocyte precursors

A

Hypocellularity

287
Q

What are the factors that can cause the leukocytosis via the mechanism of decreased margination

A
  • Exercise

- Catecholamines

288
Q

What are the common morphological features of diffuse large B cell lymphoma

A

Large cell size with a diffuse pattern of growth

-Large nuclei, open chromatin, Prominent nucleoli

289
Q

Paracortical hyperplasia is the result of which immune response

A

T cell mediated

290
Q

What is the genetic abnormality that is seen in Burkitt Lymphoma

A

-MYC

291
Q

Which LNs tend to be involved in the modular sclerosing variant of Hodgkin lymphoma

A

Lower cervical, supraclavicular, and mediastinal LNs

292
Q

What is the most common NHL

A

Diffuse large B cell lymphomas (DLBCL)

293
Q

What is Waldenstrom macroglobulinemia

A

Syndrome where the high levels of IgM leads to the high viscosity of blood

294
Q

What are the features of solitary myeloma

A

-almost inevitably progresses to multiple myeloma in 10-20 years

295
Q

What is usually the level or erythropoietin in polycythemia Vera (PCV)

A

Low, because the levels of RBCs are high

296
Q

What is the cell of origin in adult T cell leukemia/lymphoma

A

CD4 cells

297
Q

The “stary sky” appearance is indicative of which kind of general conditions

A

-rapidly growing lymphoid tissues as macrophages ingest apoptotic tumor cells

298
Q

What is the morphology of the multiple myeloma tumors

A

-Soft, gelatinous, red mass with bone lesions with punched out defects

299
Q

What is the genetic abnormality seen in polycythemia Vera

A

JAK2

300
Q

What is the chromosomal abnormality in anaplastic large cell lymphoma

A

ALK gene on 2p23

301
Q

What is the blood count finding that is indicative of chronic Myeloproliferative leukemia (CML)

A

Leukocytosis with >100,000 cells/mm3

302
Q

What is the pattern of disease in Hodgkin’s lymphoma

A
  • Starts in a single or chain of LNs

- Spreads contiguous fashion, going to the anatomically next LN

303
Q

What is the classical definition of an acute myeloid leukemia

A

-Accumulation of Immature myeloid cells (blasts) in the bone marrow that suppresses normal hematopoiesis

304
Q

Lymphoplasmacytic lymphoma has which immune markers

A

-CD20 and surface Ig

305
Q

What are the clinical features seen in primary myelofibrosis

A

-Marrow is replaced by fibrosis, leading to cytopenias and extramedulary hematopoiesis

306
Q

What is the result of chlorpromazine and phenothiazines on neutrophil counts, and what is the mechanism of action

A

Agranulocytosis due to a toxic effect on the granulocytic precursors in the bone marrow

307
Q

What is the age group commonly seen to be infected with chronic Myeloproliferative leukemia (CML)

A

Adults

308
Q

What is the underlying pathology of Burkitt’s lymphoma with regards to pathogens

A

EBV

309
Q

Which condition is commonly seen to have erythromelalgia

A

Essential thrombosis (ET), and polycythemia Vera

*which is a burning feeling in the hands and feet due to occlusion of the small arteries by pellet aggregations

310
Q

What are the morphology changes seen in mantle cell lymphoma

A
  • deeply cleave nuclear contours

- irregular nuclear outlines, condensed chromatin, scant cytoplasm

311
Q

What is the gene product affected in AML translocation t(15;17)

A

RARA (retinoic Acid receptor alpha) fuses with PML

*Commonly has a mutation in FLT3 as well

312
Q

Pulmonary langerhans cell histiocytosis develops in which patients.

A

Smokers, usually with BRAF mutations

313
Q

What conditions do patients with myelodysplastic syndromes show and what is the reasoning

A

Shows peripheral blood cytopenias, due to the abnormal cells remaining in the bone marrow

314
Q

Emboli that lodge in the spleen are most often coming from which source

A

The heart, commonly due to endocarditis of the mitral or aortic valves

315
Q

Which of the factors that cause leukocytosis via increased production in the marrow are growth factor independent

A

-Myeloproliferative disorders

316
Q

What is the treatment of ALL with the t(9;22) and what is the prognosis

A
  • chemo in addition of TK inhibitor, as (9;22) creates a BCR-ABL tyrosine kinase that is overly active
  • An Increased effectiveness in treatment
317
Q

What are the typical characteristics of lesions seen due to infection in a patients with agranulocytosis

A
  • Ulcerating necrotizing lesions of the gingeva, mouth (floor, roof, buccal, pharynx)
  • Deep undermined lesions covered by grey to green-black necrotic membranes
318
Q

Which factors of ALL imply a poorer outcome

A
  • Younger than age two, because associated with MLL gene
  • Adolescentas and adulthood presentation
  • blast count in the blood >100,000
319
Q

What is the best method for the assessment of the morphology of the hematopoietic cells

A

Marrow aspirate smears

320
Q

What is the relation in timing of antigen receptor rearrangements and malignancy

A

-Rearrangement generally precedes the malignancy, so all daughter cells share the same antigen receptor gene configuration

321
Q

What is the most common from of Hodgkin lymphoma

A

Nodular Sclerosing Type

322
Q

Which condition can increase the risk of chronic lymphocytic leukemia

A

congenital X linked agammaglobulinemia, as both has dysfunction in the BTK proteins

323
Q

What is the fastest growing tumor

A

Burkitt’s lymphoma

324
Q

What is the most common leukemia of adults in the western world

A

Chronic lymphocytic leukemia (CLL)

325
Q

What is the association between EBV and the lymphocytic rich variant of Hodgkin lymphoma

A

40%

326
Q

What are the clinical manifestations of hairy cell leukemia

A
  • Splenomegaly
  • Hepatomegaly
  • pancytopenia (due to splenic sequestration)
327
Q

What form of Reed stein burg cells are present in lymohocytic risk variant of Hodgkin lymphoma

A

Mononuclear RS cells

328
Q

What is the clinical features of Sezary syndrome

A

-skin involvment as generalized exfoliating erythroderma, rarely forming a tumor, but can have the leukemia

329
Q

What are the Extranodal sites in diffuse large B cell lymphoma that are seen

A

-GI, skin, bone, brain

330
Q

What treatment does stem cell leukemia respond to

A

PKC412 therapy

331
Q

What clinical feature can be seen lymphoplasmacytic lymphoma

A
  • IgM production leading to hyperviscosity which is known as Waldenstrom macroglobulinemia
  • secretion of red light chains and bone destruction are rare
332
Q

What are variations in the morphology of the plasma cells in multiple myeloma

A
  • Flame cells (red cytoplasms)
  • Mott cells (multiple grapelike cytoplasmic droplets)
  • Russell or Dutcher bodies (globular inclusions)
333
Q

What are the common causes of monocytosis

A
  • Chronic infections
  • Bacterial endocarditis
  • rickettsiois
  • malaria
  • IBD
334
Q

What are the histological features of the lacunar Reed-steinburg cells

A

Delicate, folded or multilobate nuclei and abundant pale cytoplasm that is disrupted during the cuttin of sections, leaving a nucleolus in an empty hole

335
Q

What are the genetic abnormalities seen in diffuse large B cell lymphomas (DLRBL)

A
  • BCL6 at 13q27 (30%)
  • BCL2 at t(14;18) (10%)
  • MYC (5%)
336
Q

In those patients that survive polycythemia Vera (PCV) for extended amounts of time end up showing which feature

A

Primary myelofibrosis with the appearance of obliterative fibrosis and extensive extra-medullary hematopoiesis (mainly the spleen)

337
Q

What are the genetic abnormalities seen with multiple myelomas

A

Translation of IgH on chromosome 14 with:

  • cyclin D1 on 11q13
  • Cyclin D3 on 6p21
338
Q

What is seen in the bone marrow cellularity in the case of megaloblastic anemia’s and myelodysplastic syndromes

A

Hypercellularity due to ineffective granulopoiesis

339
Q

What is the mutation seen in systemic mastocytosis

A

KIT

340
Q

What are the two properties of the HSC that are required

A
  • Pluripotency

- Sell renewal

341
Q

What is the classical definition of myelodysplastic syndromes

A

Defective maturation of myeloid progenitors that gives rise to ineffective hematopoiesis, leading to cytopenias

342
Q

What are the clinical presentations of hyperviscosity syndromes

A
  • Visual impairment (due to venous congestion, retinal hemorrhages)
  • Neurological problems (sluggish blood flow leading to headaches/dizzy)
  • Bleeding (formation of complexes between macroglobulins and clotting factors that interfere with platelets
  • Cyroglobulinemia (precipitation of macroglobulin at low temps leading to Raynaud)
343
Q

What is the genetic abnormality seen in chronic myelogenous leukemia (CML)

A

BCR-ABL (BCR on 22, ABL on 9) resulting in constructive ABL kinase aka, the Philadelphia translocation

344
Q

What is Bloom syndrome and which conditions are they at risk for

A
  • Genomic instability at a higher risk for cancers and acute leukemia
  • Tend to have a short stature

Robbins Pathology Heme/Lymph (Chapters 13,14) (4 decks)

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