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MS2 Unit2 > WBC disorders - Sheet1 > Flashcards

WBC disorders - Sheet1 Flashcards

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1
Q

langerhans cells are specifialized ___ cells in skin

A

dendtrici cells (for Ag presentation)

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2
Q

_____ on electron microscopy are characteristic of langerhans cell histiocytosis

A

Birbeck granules (tennis rackets, CD1a⊕ and S-100⊕)

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3
Q

pt under 2 yo presents with skin rash, cystic skeltal defects and malignant proliferation of lnagerhands cells. Dx?

A

Letterer-Siwe Dz (mult organ involvement, ± fatal)

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4
Q

adolescent pt with recent pathoglogical fracture. Biopsy shows langerhans cell swith mixed inflamm cells (↑ eosinophils). Dx?

A

eosinophilic granuloma (bening proliferation of Langerhans cells in bone)

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5
Q

pt over 3 yo presents with scalp rahs, lytic skull defect, diabetes insipidus (↑ urine output) and exophthalmos . Dx?

A

Hand-Schuller-Christian Dz

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6
Q

most common 1˚malignancy in bone dt ↑ serum IL6

A

multiple myeloma (CRAB + B sx)

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7
Q

neopasltic plasma cells in this disorder activate osteoclast ______ rcps –> lytic (punched out) bone lesions –> ↑ Ca+ & fracture risk

A

multiple myeloma/MM (RANK)

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8
Q

in MM, elevated serum proteins + M spike most commonly due to which immunoglobins

A

IgG and IgA

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9
Q

Name three features common in MM on peripheral blood smear, despotion on tissues, and urine analysis

A
  1. Rouleaux fomration (stacked RBCs);
  2. 1˚AL amyloidosis (free light chain);
  3. Bence Jones Protein (free light chain; renal failure)
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10
Q

elderly pt presents with isolated M spike on SPEP (øbone lesions, hypercalcemia, AL amyloid, Bence Jones proteinuria)

A

MGUS (pre-myeloma/dysplasia)

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11
Q

pt presents with generalized LAD, M spike, visual and neurologic deficits (dt serum hyperviscosity), and excessive bleeding (dt ∆ plt aggregation) . Dx? Tx?

A 
Waldenstrom Macroglobulinemia (↑ IgM; a large pentamer);
Tx acute sx with plasmapheresis
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12
Q

Neoplastic proliferation of cytokine secreting Reed Sternberg Cells (owl eyes)

A

Hodgkin Lymphoma (RS cells are CD15 + CD30 ⊕)

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13
Q

B sx’s for HL include (4)

A

fever, chills, wt loss, night sweats

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14
Q

HL subtypes include (4)

A
  1. Nodular sclerosis (most common)
  2. Lymphocyte rich (best px)
  3. Lymphocyte poor (worst px; HIV typical)
  4. mixed cellularity (↑ IL5 + eosinophils)
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15
Q

young female pt presents with enlarging cervical and mediastinal lymph nodes. On biopsy, lymph node has sclerotic bands and lacunar Reed Sternberg cells. Dx? Tx?

A

Nodular sclerosis subtype of HL
treat with ABVD (Doxorubicin (Adriamycin), Bleomycin, Vinblastine, dacarbazine) or Mechlorethamine (alkylating agent) and prednisone

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16
Q

this lymphoma presents in late adulthood as an enlarging lymph node or extranodal mass. its known to be clincially aggressive (high grade) and characterized a diffuse sheets of large CD20⊕ B cells

A

Diffuse large B cells lymphoma

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17
Q

young male child presents with extrandoal mass in the jaw, is sero positive for EBV and and proliferation of CD20+ cells

A

African Burkitts Lymphoma

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18
Q

young male child presents with extrandoal mass in the abdomen , is sero positive for EBV and proliferation of CD20+ cells

A

Sporadic Burkitts Lymphoma

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19
Q

Burkitt’s Lymphoma is driven by ____ translocation which leads to the overexpression of _____

A

t(8;14); c-myc + Ig heavy chain; overexpression of c-myc

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20
Q

on microscopy, Burkitt’s lymphoma can be characterized by

A

“starry sky” (high mitotic index; intermediate B cell proliferation)

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21
Q

This lymphoma is associated with chronic inflamm (eg. Hashimoto’s thryoiditis, Sjrogen Syndrome, H pylori gastritis), and formed by post-marginal center B cells

A

Marginal zone lymphoma (small B cell)

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22
Q

Gastric MALToma, lymphoma occurring at the mucosal site, is a type of ____. How do you tx?

A

Marginal zone lymphoma;

Regresses with H. pylori tx

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23
Q

Mantle Cell Lymphoma is driven by ____ translocation which leads to the overexpression of _____

A

t(11;14); Cyclin D1 + Ig heavy chain; overexpression of Cyclin D1 –> G1/S transition + proliferation

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24
Q

Older pt presents with painless LAD, small CD20+ B cell proliferation and t(11;14) translocation? Dx?

A

Mantle Cell Lymphoma (small B cell lymphoma)

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25
Older pt presents with painless LAD, small CD20+ B cell proliferation and t(14;18) translocation. Biopsy shows follicle-like nodules. Dx? Tx?
``` folicular lymphoma; tw rituximab (antiCD20) if pt is sympotmatic*** ```
26
Follicular Lymphoma is driven by ____ translocation which leads to the overexpression of _____
t(14;18); BCL2 + Ig heavy chain; overexpression of BCL2 --> apop inhibition
27
4 ways to distinguish follicular lymphoma from hyperplasia
1. abnormal lymp node structure (normal in hypreplasia) 2. ø tingible mø (reactive cells in hypreplasia) 3. Bcl2 expression (ø Bcl2 expression in hypreplasia) 4. Monoclonality (𝛋:𝛌 ratio; hyperplasia is polyclonal)
28
follicular hyperplasia is commonly seen in what type of dz states? (2)
rheuamtoid arthritis and early HIV inftn
29
painless LAD is associated with ___ (3)
chronic inflamm/lymphadenitisi, metastatic carcinoma, lymphoma
30
painful LAD is associated with
draining an acute infection (acute lymphadenitis)
31
complication of myeloporoliferative disorders include (4)
hyperuricemia + gout (↑ cell turnover) progression to fibrosis tranformation to acute leukemia
32
CML is driven by ____ translocation which leads to the overexpression of _____. Tx?
t(9;22) Ph⊕; BCR-ABL fusion --> ↑ tyrosine kinase activity; | Imatinib
33
2/3 of CML cases progress to ___ and 1/3 progress to ____
AML(60%); ALL (30%) (dt pluripotent stem cell mutation)
34
3 ways to distinguish CML from leukemoid rxn (reactive neutrophilic leukocytoiss)
1. ⊖ Leukocyte Alkaline Phosphatase (⊕ in leukamoid granulocytes) 2. ↑ basophils (ø in leukamoid) 3. Ph⊕ (⊖ in LR)
35
CML has a characteristic proliferation of what cell types?
mature myeloids esp granulocytes + basophils
36
p. vera has a characteristic proliferation of what cell types?
mature myeloids esp RBCs***, granulocytes, plts
37
p.vera is driven by ____ mutation which leads to what clinical finding?
JAK2 kinase mutation; hyperviscous blood
38
Hypervisous blood in p.vera has what assc sx? (4)
1. blurry vision + headache 2. venous thrmobosis (budd Chiari hepatic vein*) 3. flushed face (plethora dt congestion) 4. itching after hot bath (↑ histamine + mast cells)
39
Treat p. vera with? (3)
1. phlebotomy 2. hydroxyurea 3. ruxolitinib otw death in a year
40
3 ways to distinguish PV from reactive polycythemia (in PV/high altitude/ectopic renal cell carcinoma)
1. PV: ↓EPO + normal SaO2 2. reactive poly dt high altitude/lung dz: ↑EPO + low SaO2 3. reactive ectopic renal cell carcinoma: ↑EPO + normal SaO2
41
essential thrombocythemia (ET) is driven by ____ mutation which leads to what clinical finding?
JAK2 kinase mutation; ↑risk for bleeding/thrombrosis (ø hyperuricemia/gout/progression to marrow fibrosis or acute leukemia)
42
ET has a characteristic proliferation of what cell types?
mature myeloids esp plts***, granulocytes, RBCs
43
Treat ET with? (3)
Angrelide (from TBL), hydroxyurea, ruxolitinib (JAK2 kinase inhibitor)
44
Myelofibrosis has a characteristic proliferation of what cell types?
mature myeloids esp MGKs
45
myelofibrosis is driven by ____ mutation which leads to what clinical finding?
JAK2 kinase mutation (50%); ↑PDGF causing fibrosis (↓hematopoiesis --> ↑ risk for inftn, thrombosis, and bleeding)
46
Older pt presents with splenomegaly, leukoerythoblastic smear and and ↑ PDGFexpression. Dx? Pt is at risk for (3) ? How do you Tx?
- myelofibrosis (tear drop/nucleated RBCs + immature granulocytes); - inftn; thrombosis, bleeding - tw Ruxolitinib (JAK2 inh)
47
splenomegaly in myelofibrosis is dt ____
extramedullary hematopoeisis
48
↑ of mature CD+ T cells that progress to sezary cells in blood or infiltrate the skin (pautrier microabscesses). Pt presents with rash, plaques, and nodules
Mycosis fungoides
49
in Mycosis Fungoides cells can spread to the blood, producing ______
Sezary Syndrome (cerebriform nucleated cells)
50
↑ of mature CD+ T associated with HTLV-1. Pt presents with rash, LAD, hepatosplenomegaly, and lytic bone lesions (↑Ca+)
Adult T Cell Leukemia/Lymphoma ATLL (HTLV-1common in Japan and Caribbean; ATLL ≠ MM (ø AL or renal sx like CRAB)
51
↑ of mature B cells positive for tartrate resistance acid phosphatase (TRAP+).
Hair Cell Leukemia
52
Pt presents with ⊕ TRAP, dry tap on bone marrow aspiration, and splenomegaly, ⊖LAD. Dx? Tx?
``` Hairy Cell Leukemia (dry tap = fibrosis; splenomegaly = ↑ trapped hair cells) Tw cladribine (2CDA = adenosine deaminase inh, stops toxic acc'm) ```
53
↑ of naive CD5 and CD20 B cells. Pt presents with generalize LAD and/or small lyphocytic lymphoma. Dx? Tx?
CLL | observation --> rituximab + accumPemrbrolizumab (antiPDL1)
54
smudge cells are characteristic of?
CLL
55
3 complications of CLL
1. Hypogammaglobulineamia (death by inftn) 2. Autoimmune hemolytic anemia 3. Richter Transformation (to diffuse large Bcell lymphoma; enlarging lymph node or spleen)
56
Crystal aggregates of _____ are seen as ______ in Acute Myeolid Leukemia (ALL)
myeloperoxidase (MPO); Auer Rods
57
what kind of syndromes present with cytopenias, hypercellular bone marrow, abnormal maturation of cells, and increased blast (<20%)
myelodysplastic syndromes
58
_____ is characterized by proliferation of MGKs and ø MPO; also assc with down syndrome prior to 5 yo
Acute Megakaryoblastic leukemia (AML subtype)
59
_____ is characteriszed by proliferation of monocytes and ø MPO; blasts infiltrate gums
Acute Monocytic Leukema
60
Acute Promyelocytic Leukema is driven by ____ translocation which leads to _____. Tx?
``` t(15;17) retinoic acid rcp tranlocation --> RAR disruption + ↑ blast accumulation tw ATRA (vitA derivative --> blast maturation) ```
61
APL pts are at risk for what emergent complication
DIC (↑ primary granules)
62
>20% blasts characterizes
acute leukemia
63
↑ acc'm of TdT⊕ lymphocytes; assoc with Down syndrome in pts under 5yo
Acute Lymphoblastic leukemia (T-ALL vs B-ALL)
64
Teenage pt presents with thymic (mediastinal) mass and TdT⊕ lymphoblasts with ⊕CD2,3,4,7,8 and CD10⊖. Dx?
T-ALL
65
Child pt presents with t(12;21)⊕ cytogenetic test and TdT⊕ lymphoblasts with ⊕CD10, 19, 20 and
B-ALL (good px)
66
Elderly pt presents with t(9;22)⊕ cytogenetic test and TdT⊕ lymphoblasts with ⊕CD10, 19, 20 and
Ph+ ALL (B-ALL; poor px)

MS2 Unit2 (8 decks)

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