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Skin, Blood & Lymph Exam #1 > WBC Pathology 3 > Flashcards

WBC Pathology 3 Flashcards

1
Q

What is the most common and deadly plasma cell neoplasm?

A

Multiple myeloma

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2
Q

What is needed for diagnosis of multiple myeloma?

What is seen in the bone marrow?

A

Radiological and laboratory findings; definitive diagnosis requires a BM biopsy.

The BM contains > 30% plasma cells with considerable atypia.
There is often > 3 g/dL of Ig (M protein) and/or > 6 g/dL of urinary Bence-Jones proteins.

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3
Q

What is the presentation and prognosis of solitary myeloma (plasmacytoma)?

A

It presents as a single mass in bone or soft tissue. Solitary intraosseous plasmacytomas (most common) have an aggressive progression and almost always progresses to MM within 10-20 years

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4
Q

What is the presentation and characteristic finding in smoldering myeloma?

A

There tends to be a lack of symptoms and a high serum M component (>3 g/dL). In about 75% of patients, it progresses to M over 15 years.

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5
Q

What site(s) dominate in multiple myeloma?

A

Primarily it is a bony disease, but may spread to the LNs and extranodal sites late in its course.

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6
Q

What is the most common “patient-type” of multiple myeloma?

A

M>F and with increased incidence in African patients.

It is a disease of older adults (peak age is 65-70 y/o).

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7
Q

What is elevated in the blood and urine in 99% of patients with multiple myeloma?

What other unique marker is elevated?

A

Increased Igs in the blood - M protein (IgG) and IgA.
-3g/dL of serum Ig.
Bence-Jones proteins in the urine.
-6m/dL in urine.

B2-microglobulin: non-specific and not used in diagnosis, but may be useful in prognosis.

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8
Q

Why is hyperviscosity noted in 7% of patients with multiple myeloma?

A

Because there is excessive production and aggregation of M proteins, usually IgA and IgG3 subtypes.

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9
Q

Does absence of M protein exclude multiple myeloma?

A

No, about 1% of MM is non-secretory.

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10
Q

What is the immunophenotype of multiple myeloma? (2)

A

+ CD138 (syndecan-1)

+ CD56

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11
Q

What are the clinical features of multiple myeloma? (4)

A

Lytic bone lesions throughout the skeletal system.

Suppression of normal humoral immunity, which leads to recurrent bacterial infections.

Bone resorption which leads to pathologic fractures and hypercalcemia (confusion, weakness, lethargy, constipation, polyuria, etc.).

Renal failure due to Bence-Jones proteinuria, as these proteins are toxic to renal tubular cells. It may also cause amyloidosis in renal tissue.

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12
Q

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

A

Bacterial infections trailed by renal failure.

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13
Q

What is considered to be the most common plasma cell disorder?

A

Monoclonal gammopathy of uncertain (clinical) significance (MGUS).

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14
Q

At what age is MGUS most common?

A

It occurs in 3% of people >50 y/o and 5% in those >70 y/o.

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15
Q

What is the presentation of MGUS?

What is the progression of it?

A

It presents asymptomatically and with an [M protein] < 3 g/dL.

1% of patients with MGUS will convert to MM, so serum M protein and Bence-Jones proteinuria must be monitored over time.

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16
Q

What is the connection between MGUS and MM?

A

They both share some of the same chromosomal translocations and deletions.

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17
Q

What age is lymphoplasmacytic lymphoma most often diagnosed?

What is the pathogenesis of it?

How is it different from MM symptomatically?

A

6th or 7th decade of life.

It is a B-cell neoplasm and bears a superficial resemblance to CLL/SLL, but it has a substantial fraction of cells that undergo terminal differentiation to plasma cells.

It does not have any features of RF or lytic bone lesions.

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18
Q

What is Waldenstrom macroglobinemia?

A

A process that occurs in lymphoplasmacytic lymphoma where the plasma cell component secretes enough IgM to cause hyperviscosity.

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19
Q

What mutation is seen in virtually all cases of lymphoplasmacytic lymphoma?

A

MYD88

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20
Q

What are symptoms of hyperviscosity syndrome?

A

Retinopathy (visual changes)

Neurological symptoms (ranging from HA/vertigo to SZ and coma)

Spontaneous bleeding

*there is a spectrum of symptoms caused by this.

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21
Q

What is the presentation of mantle cell lymphoma?

What is the progression?

Which variant is associated with an even worse prognosis?

A

Usually presents as painless lymphadenopathy, but may have symptoms related to spleen and gut involvement.

Poor prognosis with a mean survival of 3-4 years. It is not curable, but HSC transplant has showed some promise.

The blastoid variant and a “proliferative” expression.

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22
Q

What malignancy is associated with a (11;14) translocation involving IgH and cyclin D1?

A

Mantle cell lymphoma

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23
Q

What is a unique histological finding in mantle cell lymphoma?

A

Lymphomatoid polyposis

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24
Q

Where do marginal zone lymphomas occur?

What is the progression?

A

They arise in sites exhibiting chronic inflammation of autoimmune or infectious etiology (ex: Sjogren syndrome, H. pylori, Hashimoto’s, etc.).
AKA “maltomas”.

They tend to remainlocalized for prolonged periods and may regress if the inciting agent(s) are eliminated.

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25
Q

How do marginal zone lymphomas begin?

A

They begin “polyclonal”.

26
Q

The theme of polyclonal to monoclonal transformation during lymphomagenesis is applicable to what?

A

EBV-associated or associated lympomas.

27
Q

Who is most likely to be diagnosed with hairy cell leukemia?

Upon attempting to make a diagnosis, what may impair you?

A

It is 5x more common in men and the average age is 55 y/o (“middle-aged white males”).

BM aspiration is often hard, resulting in a “dry tap”.

28
Q

What is the presentation of hairy cell leukemia?

What is a unique association?

What is the progression?

A

Splenomegaly and pancytopenia (due to BM and spleen involvement).

1/3 are association with infections, with an increased incidence of atypical mycobacterial infections.

It is generally indolent and the outlook is great.

29
Q

What mutation is associated with hairy cell leukemia?

A

BRAF activation

30
Q

What is the common presentation of peripheral T-cell lymphoma, unspecified?

What is the prognosis?

What is the manner of diagnosis?

A

Lymphadenopathy sometimes with eosinophila, pruritis, fever and weight loss.

The prognosis is poor (much worse than B-cell analogues).

Immunophenotyping.

31
Q

What is the presentation of anaplastic large cell lymphoma?

What is the prognosis?

What is used in diagnosis? Why?

What is the morphology?

A

Soft tissue masses in kids or young adults.

Prognosis is good.

ALK rearrangements, because ALK should not be expressed in normal lymphocytes. It is also CD30+.

The tumor cells tend to cluster around venules and infiltrate lymphoid sinuses - mimics metastatic carcinoma.

32
Q

What neoplasm is associated with HTLV-1 infection?

A

Adult T-cell leukemia/lymphoma

33
Q

What is the presentation of large granular lymphocytic leukemia?

What is the histological finding?

A

Neutropenia and anemia are common (even though there is little BM involvement) and may be associated with Feltys syndrome.

Tumor cells are large lymphocytes with blue cytoplasm and scant coarse azurophilic granules.

34
Q

What is associated with extranodal NK/T-cell lymphoma?

How does it present?

A

EBV.

Typically presents as a highly destructive nasopharyngeal mass; less often involves the testes and skin.

35
Q

Define the following:

Acute myeloid leukemias (AML)

Myeloidysplastic syndrome (MDS)

Myeloproliferative disorders (MPD)

A

Acute myeloid leukemias (AML): accumulation of blasts in BM suppresses normal hematopoiesis (>20% blasts in BM).

Myeloidysplastic syndrome (MDS): ineffective hematopoiesis leads to cytopenias.

Myeloproliferative disorders (MPD): increased production of one or more types of committed/mature white/red cells of the myeloid series.

*MDS and MPD often transform into AML.

36
Q

What are the following markers found on?

CD34
CD64
CD33
CD15

A

CD34 - precursor/immature myeloid
CD64 - mature myeloid
CD33 - myeloid progenitors and monocytes
CD15 - granulocytes/RS cells and variants

37
Q

What is the presentation/clinical features of AML? (4)

A

Fatigue (anemia), fever/infection (neutropenia), bleeding (thrombocyopenia).

Petechiae/ecchymoses/mucosal hemorrhages/hematuria.

Infections (fungi, Pseudomonas, commensals, Pnemocystis, etc.)

Tissue infiltration (skin, gingiva, eyes), especially with monocytic infiltration.

38
Q

What is the primary tumor mass called in AML?

Do CNS spread occur?

A

“Granulocytic sarcoma”

Yes, but less often than ALL.

39
Q

What causes MDS?

A

Idiopathic

Secondary - due to prior genotoxic drug or radiation therapy (worse prognosis)

40
Q

Who is most likely to get MDS?

How frequently does it transform?

A

Older adults (mean 70 y/o).

10-40% of the time, but more common in those with t-MDS (survival only 4-8 mo.).

41
Q

What mutations are associated with following MPDs?

Chronic myeloid leukemia
Polycythemia vera
Essential thrombocytosis

A

Chronic myeloid leukemia: BCR-ABL fusion

Polycythemia vera: JAK2 point mutations

Essential thrombocytosis: JAK2 point mutations

42
Q

EMH is common in which disorders?

A

MPDs

43
Q

What chromosomal abnormality is seen in chronic myeloid leukeimia (CML)?

A

BCR-ABL - (9;22)(q34:q11) on Philadelphia chromosome

44
Q

What age is most common for CML?

What is the presentation?

What is used for diagnosis? (3)

A

M>F; average age is 50 y/o.

Onset is insidious - patients may have fatigue, weight loss, anorexia, and abdominal fullness (due to splenomegaly EMH).

Leukocytosis (>100K ct with immature forms and <10% blasts).
BM is hypercellular.
Detection of BCR-ABL.

45
Q

What enzyme level is low in CML?

A

Leukocyte alkaline phosphatase (LAP)

46
Q

What is the prognosis of CML if untreated?

What happens to about 50% of patients?

A

It has a slow progression with moderate anemia and hypermetabolism. 3 year survival rate without treatment.

50% of patients enter an accelerated phase after 6-12 mo. and enter a “blast crisis” (i.e. acute leukemia) - most commonly AML-like, but some are ALL-like.
The other 50% will enter a “blast crisis” without going though the accelerated phase.

47
Q

What is the level of EPO in polycythemia vera (MPD)?

A

Low, due to elevated RBCs

48
Q

What is the presentation of PV?

What is the Hct.?

Many patients come to clinical attention due to what?

A

Insidious; plethora, cyanosis, pruritis, HA, HTN, GI ulceration, gout.

> 55% Hct.

DVT, MI, stroke.

49
Q

What are treatment options for PV?

How common is progression to AML?

A

Phlebotomy + JAK2 inhibitors

Only 1-2% progress to AML.

50
Q

What change is seen in the BM of a patient with PV?

A

Marrow fibrosis

51
Q

What is the molecular change seen in essential thrombocytosis (ET)?

A

Activating mutations in JAK2, which would normally be activated by TPO.

52
Q

What is the presentation of ET?

How is it diagnosed?

What is the prognosis?

Does it progress to AML?

A

It is insidious with few symptoms. There will be bleeding/clotting complicatins.

BM biopsy is needed, but it is a diagnosis of exclusion because all chronic MPDs have elements of thrombocytosis.

Median survival of 12-15 years.

No.

53
Q

What is the “hallmark” of primary myelofibrosis?
How does this happen?

What mutations are common?

What are the symptoms?

What is the progression and usual cause of death?

A

The development of obliterative marrow fibrosis by non-neoplastic fibroblasts. This leads to cytopenias and EMH.
This occurs due to the abnormal megakaryocytes producing lots of PDGF and TGF-b.

JAK2 mutations are common.

Splenomegaly, fatigue and normocytic, normochromic anemia.

Typically 3-5 years, death resulting from complications of cytopenias (infections, thrombosis, bleeding, etc.).

54
Q

In primary myelofibrosis, if marrow fibrosis is prominent enough, what may occur?

A

AML at extramedullary sites, including LNs and soft tissues.

55
Q

What 3 cell markers are positive on Langerhans cells?

A

S100
CD1a
HLA-DR

56
Q

What is a classic histological finding Langerhans cell histiocytosis?

A

Birbeck granules in the cytoplasm

57
Q

What age is most likely to be diagnosed with multifocal/multisystemic LCH (Letterer-Siwe disease)?

What are the symptoms/presentation? (4)

What is the prognosis?

A

< 2 y/o.

Cutaneous lesions (look like seborrheic eruptions)
Fever, infections, OM
HSM, lymphaenopathy
Pulmonary and bone lesions

50% 5-year survival with aggressive chemo; otherwise fatal.

58
Q

What symptoms are seen in unifocal and multifocal unisystem LCH (eosinophilc granuloma)?

What is the treatment?

A

Unifocal: skeletal system in older kids.
-calvarium, rube and femur.

Multifocal unisystem: multiple bony masses in young kids.
-DI in 50%

Chemo if multifocal. If unifocal, local excision or regression. Patients may have spontaneous regression.

59
Q

What is the Hand-Schuller-Christian triad in multifocal unisystem LCH?

A

Calvarial bone defects
DI
Exophthalmos

60
Q

How does pulmonary LCH present?

It may be a recative/inflammatory process, but it is considered neoplastic if the patients have what mutations?

What is the major association?

A

BL interstitial disease - multiple fine nodules and cysts in the mid and upper lung zones.

BRAF mutations.

Smoking - may regress if smoking is ceased.

Skin, Blood & Lymph Exam #1 (12 decks)

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