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MALIGNANT HEME > SET12 > Flashcards

SET12 Flashcards

1
Q

What mutation has been seen in 50% of Langerhans Histiocytosis and in 50-100% of patients with Erdheim-Chester disease

A

BRAF V600E - Vemurafenib useful; Erdheim Chester is a non-Langerhans histiocytosis

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2
Q

What is a good way to differentiate Mantle Cell from SLL/CLL?

A

CD23 positivity (CD23 is a B Cell Activation antigen)

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3
Q

How do you manage Sezary Syndrome vs. Mycosis Fungoides?

A

Sezary syndrome requires both systemic AND topical treatment (oral retinoids like Bexarotene; HDAC inhibitors like Romidepsin; INF-alph, MTX, and extracorporeal photopheresis)

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4
Q

What is unique about the extranodal nasal type NK/T cell lymphoma?

A

It often has absence of T cell antigens and TCR gene rearrangements

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5
Q

Why can Ibrutinib be useful for Waldenstroms?

A

Because the MYD88 mutation leads to signaling via Bruton’s Tyrosine Kinase so that there is a targeted option; Ibrutinib 420 mg daily is 2nd line tx

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6
Q

What is Monoclonal B-Cell Lymphocytosis?

A

essentially it is pre-CLL i.e. clonal but w/ ALC <5k

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7
Q

What virus is implicated in Adult T Cell Leukemia/lymphoma

A

HTLV-1- often has osteolytic lesions leading to hypercalcemia

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8
Q

What is the more aggressive variant of mantle cell lymphoma?

A

Blastoid Variant

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9
Q

What is the standard of care for PTLD?

A

Withdrawing the immunosuppression a bit and then if that fails–> Rituximab

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10
Q

What is CD30 ?

A

It is a TNF-alpha receptor and is expressed in ALCL and in classical Hodgkin Reed-Sternberg Cells

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11
Q

Where does Endemic Burkitt often occur? Sporadic Burkitt?

A

Endemic is often in extranodal sites such as the jaw, testes, ovaries, kidneys; Sporadic is often in the abdomen

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12
Q

What is responsible for the “Starry Sky” appearance on Burkitt Bx?

A

Dead tumor in macrophages leads to stars in a sky of tumor

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13
Q

What is the management of Unicentric Castleman Disease?

A

Simply removing the lymph node

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14
Q

What is the pathophysiology of Burkitt Lymphoma?

A

Very aggressive lymphoma w/ t(8;14) leading to c-myc being translocated near the IgH locus w/ overexpression

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15
Q

What is the characteristic cell found in the blood of Adult T Cell Leukemia (Assoc. HTLV-1)

A

Flower Cells

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16
Q

Thyroid MALT is associated with ______

A

Hashimoto thyroiditis

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17
Q

What are the 4 independent MIPI factors (mantle IPI)?

A

Age, Performance Status, LDH, and WBC count

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18
Q

What is the significance of ALK in anaplastic Large Cell Lymphoma (ALCL)?

A

ALK positivity confers a better prognosis than ALK (anaplastic lymphoma kinase) negative

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19
Q

What is the etiology of Primary Effusion Lymphoma?

A

It is a lymphoma associated with HIV and the driver is HHV8 (recall HHV8 also causes Kaposi Sarcoma)

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20
Q

What is Denileukin Difitox?

A

Engineered protein that combines IL-2 with the diphtheria toxin and binds to CTCL (Cutaneous T Cell Lymphoma) cells at the CD25 component of the IL-2 receptor

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21
Q

Do both ALK (+) and ALK (-) anaplastic large cell lymphomas get treated with Brentuximab Vedotin?

A

Yes both are CD30 positive

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22
Q

What is the MOA of Brentuximab vedotin? What are two major cases it is used in?

A

Targets CD30 receptor and useful in Relapsed Hodgkins (I think maybe even straight up in some now) and in Anaplastic Large Cell Lymphoma

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23
Q

Ocular-Adnexal MALT is associated with _____

A

Chlamydophila psittaci

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24
Q

What is vorinostat?

A

A medication approved for patients with cutaneous T cell lymphomas who have failed two systemic therapies

25
Q

CD56 is a marker for _____

A

NK cells

26
Q

What virus driven lymphoma often presents with hypercalcemia due to osteolytic lesions?

A

Adult T Cell Leukemia/Lymphoma (HTLV-1)

27
Q

Primary Effusion Lymphoma and Plasmablastic Lymphoma occur most commonly in what population?

A

HIV/AIDS

28
Q

What is often the treatment for Natural Killer Cell lymphomas

A

concurrent chemo and radiation i.e. SMILE regimen (Dex, MTX, Ifosfamide, L-asparaginase, and Etoposide)

29
Q

Stomach MALT is associated with ______

A

H. pylori

30
Q

What is a major difference in flow for follicular and mantle cell?

A

Follicular is CD10+

31
Q

Sezary Syndrome affects these cells _____

A

CD4 positive T cells (leukemic variant of mycosis fungoides)

32
Q

MYD88 is useful for what

A

Differentiatiing Waldenstroms from other B cell disorders; MYD88 leads to signalling through Bruton’s Tyrosine Kinase (Ibrutinib useful here)

33
Q

Studies have shown that DLBCL remission at ______ years portends a good prognosis

A

Two years

34
Q

What is the mgmt of relapsed DLBCL?

A

They should get some sort of second line (salvage) therapy (R-ICE; R-ESHAP; R-DHAP) and then should be consolidated by an autologous transplant

35
Q

What is the treatment for limited stage non-bulky DLBCL vs. limited stage bulky DLBCL?

A

Limited stage = Stage I or II (Bulky = LN > 10 cm); non bulky = 3 CY R-CHOP + ISRT; bulky limited = 6 CY R-CHOP +ISRT

36
Q

What are two types of Primary Cutaneous CD30 + lymphoproliferative disorders (good tx?)

A

Lymphomatoid papulosis and Primary Anaplastic Large Cell lymphoma - you would THINK it should be Brentuximab Vedotin but really the Tx here is surgery; note that this is NOT the case w/ Mycosis fungoides

37
Q

Small bowl MALT is associated with _______

A

C. jejuni

38
Q

Another name for Castleman Disease

A

Angiofollicular Lymph Node Hyperplasia

39
Q

This represents over 60% of cutaneous T cell lymphomas

A

Mycosis fungoides (CD4 cell as cell of origin-COO)

40
Q

What is unique about primary mediastinal B cell lymphoma?

A

It is a subtype of DLBCL that shares features with Nodular Sclerosing Hodgkin Lymphoma

41
Q

What is “Mature B-ALL”?

A

Burkitt Leukemia

42
Q

What is a backbone of tx for T cell prolymphocytic leukemia?

A

Alemtuzumab (check for CMV reactivation) as they are often Tdt-, CD1a-, CD2+, CD7+, and CD52+ (Alemtuzumab/Campath is anti-CD52)

43
Q

How can Multicentric Castleman Disease present?

A

It is a lymphoproliferative disorder and can lead to hyperactivation of the immune system with excessive cytokines and MODS

44
Q

How do you treat ocular adnexal MALT lymphoma?

A

Doxycycline as the cause is C. psittaci; this is just like tx Gastric MALT w/ triple therapy

45
Q

What is the NK cell marker?

A

CD56

46
Q

What B cell disorder is assoc with SOLID organ transplant

A

PTLD (Post-Transplant Lymphoproliferative Disorder) , often EBV +

47
Q

What is “limited stage” DLBCL?

A

Stage I or II

48
Q

What mutation confers Ibrutinib resistance in Waldenstroms?

A

CXCR4

49
Q

How do you manage mycosis fungoides with limited skin involvement?

A

topical retinoids (Bexarotene), phototherapy (PUVA), UVB, topical imiquimod, topical chemo (carmustine)

50
Q

What is the concern for AIDS pts who get Rituximab?

A

CD4 count less than 100 may increase the risk of infections

51
Q

What is the definition of Sezary Syndrome?

A

Clonal TCR rearrangement AND either 1000/mcl Sezary cells OR increase CD4 or CD3 cells with a CD4/CD8 ratio > 10

52
Q

What is BEAM?

A

A regimen for Auto-Transplant i.e. possibly in DLBCL- Carmustine (BCNU), Etoposide, Ara-C, and Melphalan

53
Q

What are the 3 types of Burkitt Lymphoma?

A

1) Endemic Burkitt i.e. EBV associated and in Africa; Sporadic Burkitt (less common US cause) 3) Immunodeficiency (HIV) most common cause in US

54
Q

Parotid MALT is associated with _______

A

Sjogren syndrome

55
Q

Which T/NK cell lymphomas often have absence of T cell antigens and TCR gene rearrangements?

A

Extranodal Nasal-Type NK/T cell lymphoma

56
Q

What is a translocation commonly seen in ALCL?

A

t(2;5)

57
Q

How do you treat mycosis fungoides with generalized skin involvement?

A

Total Skin Electron Beam Tx

58
Q

This gene mutation is useful for differentiating Waldenstroms (Lymphoplasmacytic Lymphoma) from other B cell disorders

A

MYD88 = Myeloid Differentiation Primary Response 88 (strange bc is B cell not myeloid)

59
Q

What is Lymphomatoid Granulomatosis?

A

a rare EBV-associated lymphoproliferative disorder often involving the lungs in a nodular pattern

MALIGNANT HEME (12 decks)

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