SET12 Flashcards
What mutation has been seen in 50% of Langerhans Histiocytosis and in 50-100% of patients with Erdheim-Chester disease
BRAF V600E - Vemurafenib useful; Erdheim Chester is a non-Langerhans histiocytosis
What is a good way to differentiate Mantle Cell from SLL/CLL?
CD23 positivity (CD23 is a B Cell Activation antigen)
How do you manage Sezary Syndrome vs. Mycosis Fungoides?
Sezary syndrome requires both systemic AND topical treatment (oral retinoids like Bexarotene; HDAC inhibitors like Romidepsin; INF-alph, MTX, and extracorporeal photopheresis)
What is unique about the extranodal nasal type NK/T cell lymphoma?
It often has absence of T cell antigens and TCR gene rearrangements
Why can Ibrutinib be useful for Waldenstroms?
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
What is Monoclonal B-Cell Lymphocytosis?
essentially it is pre-CLL i.e. clonal but w/ ALC <5k
What virus is implicated in Adult T Cell Leukemia/lymphoma
HTLV-1- often has osteolytic lesions leading to hypercalcemia
What is the more aggressive variant of mantle cell lymphoma?
Blastoid Variant
What is the standard of care for PTLD?
Withdrawing the immunosuppression a bit and then if that fails–> Rituximab
What is CD30 ?
It is a TNF-alpha receptor and is expressed in ALCL and in classical Hodgkin Reed-Sternberg Cells
Where does Endemic Burkitt often occur? Sporadic Burkitt?
Endemic is often in extranodal sites such as the jaw, testes, ovaries, kidneys; Sporadic is often in the abdomen
What is responsible for the “Starry Sky” appearance on Burkitt Bx?
Dead tumor in macrophages leads to stars in a sky of tumor
What is the management of Unicentric Castleman Disease?
Simply removing the lymph node
What is the pathophysiology of Burkitt Lymphoma?
Very aggressive lymphoma w/ t(8;14) leading to c-myc being translocated near the IgH locus w/ overexpression
What is the characteristic cell found in the blood of Adult T Cell Leukemia (Assoc. HTLV-1)
Flower Cells
Thyroid MALT is associated with ______
Hashimoto thyroiditis
What are the 4 independent MIPI factors (mantle IPI)?
Age, Performance Status, LDH, and WBC count
What is the significance of ALK in anaplastic Large Cell Lymphoma (ALCL)?
ALK positivity confers a better prognosis than ALK (anaplastic lymphoma kinase) negative
What is the etiology of Primary Effusion Lymphoma?
It is a lymphoma associated with HIV and the driver is HHV8 (recall HHV8 also causes Kaposi Sarcoma)
What is Denileukin Difitox?
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
Do both ALK (+) and ALK (-) anaplastic large cell lymphomas get treated with Brentuximab Vedotin?
Yes both are CD30 positive
What is the MOA of Brentuximab vedotin? What are two major cases it is used in?
Targets CD30 receptor and useful in Relapsed Hodgkins (I think maybe even straight up in some now) and in Anaplastic Large Cell Lymphoma
Ocular-Adnexal MALT is associated with _____
Chlamydophila psittaci
What is vorinostat?
A medication approved for patients with cutaneous T cell lymphomas who have failed two systemic therapies
CD56 is a marker for _____
NK cells
What virus driven lymphoma often presents with hypercalcemia due to osteolytic lesions?
Adult T Cell Leukemia/Lymphoma (HTLV-1)
Primary Effusion Lymphoma and Plasmablastic Lymphoma occur most commonly in what population?
HIV/AIDS
What is often the treatment for Natural Killer Cell lymphomas
concurrent chemo and radiation i.e. SMILE regimen (Dex, MTX, Ifosfamide, L-asparaginase, and Etoposide)
Stomach MALT is associated with ______
H. pylori
What is a major difference in flow for follicular and mantle cell?
Follicular is CD10+
Sezary Syndrome affects these cells _____
CD4 positive T cells (leukemic variant of mycosis fungoides)
MYD88 is useful for what
Differentiatiing Waldenstroms from other B cell disorders; MYD88 leads to signalling through Bruton’s Tyrosine Kinase (Ibrutinib useful here)
Studies have shown that DLBCL remission at ______ years portends a good prognosis
Two years
What is the mgmt of relapsed DLBCL?
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
What is the treatment for limited stage non-bulky DLBCL vs. limited stage bulky DLBCL?
Limited stage = Stage I or II (Bulky = LN > 10 cm); non bulky = 3 CY R-CHOP + ISRT; bulky limited = 6 CY R-CHOP +ISRT
What are two types of Primary Cutaneous CD30 + lymphoproliferative disorders (good tx?)
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
Small bowl MALT is associated with _______
C. jejuni
Another name for Castleman Disease
Angiofollicular Lymph Node Hyperplasia
This represents over 60% of cutaneous T cell lymphomas
Mycosis fungoides (CD4 cell as cell of origin-COO)
What is unique about primary mediastinal B cell lymphoma?
It is a subtype of DLBCL that shares features with Nodular Sclerosing Hodgkin Lymphoma
What is “Mature B-ALL”?
Burkitt Leukemia
What is a backbone of tx for T cell prolymphocytic leukemia?
Alemtuzumab (check for CMV reactivation) as they are often Tdt-, CD1a-, CD2+, CD7+, and CD52+ (Alemtuzumab/Campath is anti-CD52)
How can Multicentric Castleman Disease present?
It is a lymphoproliferative disorder and can lead to hyperactivation of the immune system with excessive cytokines and MODS
How do you treat ocular adnexal MALT lymphoma?
Doxycycline as the cause is C. psittaci; this is just like tx Gastric MALT w/ triple therapy
What is the NK cell marker?
CD56
What B cell disorder is assoc with SOLID organ transplant
PTLD (Post-Transplant Lymphoproliferative Disorder) , often EBV +
What is “limited stage” DLBCL?
Stage I or II
What mutation confers Ibrutinib resistance in Waldenstroms?
CXCR4
How do you manage mycosis fungoides with limited skin involvement?
topical retinoids (Bexarotene), phototherapy (PUVA), UVB, topical imiquimod, topical chemo (carmustine)
What is the concern for AIDS pts who get Rituximab?
CD4 count less than 100 may increase the risk of infections
What is the definition of Sezary Syndrome?
Clonal TCR rearrangement AND either 1000/mcl Sezary cells OR increase CD4 or CD3 cells with a CD4/CD8 ratio > 10
What is BEAM?
A regimen for Auto-Transplant i.e. possibly in DLBCL- Carmustine (BCNU), Etoposide, Ara-C, and Melphalan
What are the 3 types of Burkitt Lymphoma?
1) Endemic Burkitt i.e. EBV associated and in Africa; Sporadic Burkitt (less common US cause) 3) Immunodeficiency (HIV) most common cause in US
Parotid MALT is associated with _______
Sjogren syndrome
Which T/NK cell lymphomas often have absence of T cell antigens and TCR gene rearrangements?
Extranodal Nasal-Type NK/T cell lymphoma
What is a translocation commonly seen in ALCL?
t(2;5)
How do you treat mycosis fungoides with generalized skin involvement?
Total Skin Electron Beam Tx
This gene mutation is useful for differentiating Waldenstroms (Lymphoplasmacytic Lymphoma) from other B cell disorders
MYD88 = Myeloid Differentiation Primary Response 88 (strange bc is B cell not myeloid)
What is Lymphomatoid Granulomatosis?
a rare EBV-associated lymphoproliferative disorder often involving the lungs in a nodular pattern
