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MS2 Unit2 > WBC disorders - Sheet1 > Flashcards

WBC disorders - Sheet1 Flashcards

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
Q

Older pt presents with painless LAD, small CD20+ B cell proliferation and t(14;18) translocation. Biopsy shows follicle-like nodules. Dx? Tx?

A 
folicular lymphoma;
tw rituximab (antiCD20) if pt is sympotmatic***
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26
Q

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

A

t(14;18); BCL2 + Ig heavy chain; overexpression of BCL2 –> apop inhibition

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27
Q

4 ways to distinguish follicular lymphoma from hyperplasia

A
  1. abnormal lymp node structure (normal in hypreplasia)
  2. ø tingible mø (reactive cells in hypreplasia)
  3. Bcl2 expression (øBcl2 expression in hypreplasia)
  4. Monoclonality (𝛋:𝛌 ratio; hyperplasia is polyclonal)
28
Q

follicular hyperplasia is commonly seen in what type of dz states? (2)

A

rheuamtoid arthritis and early HIV inftn

29
Q

painless LAD is associated with ___ (3)

A

chronic inflamm/lymphadenitisi, metastatic carcinoma, lymphoma

30
Q

painful LAD is associated with

A

draining an acute infection (acute lymphadenitis)

31
Q

complication of myeloporoliferative disorders include (4)

A

hyperuricemia + gout (↑ cell turnover)
progression to fibrosis
tranformation to acute leukemia

32
Q

CML is driven by ____ translocation which leads to the overexpression of _____. Tx?

A

t(9;22) Ph⊕; BCR-ABL fusion –> ↑ tyrosine kinase activity;

Imatinib

33
Q

2/3 of CML cases progress to ___ and 1/3 progress to ____

A

AML(60%); ALL (30%) (dt pluripotent stem cell mutation)

34
Q

3 ways to distinguish CML from leukemoid rxn (reactive neutrophilic leukocytoiss)

A
  1. ⊖ Leukocyte Alkaline Phosphatase (⊕ in leukamoid granulocytes)
  2. ↑ basophils (ø in leukamoid)
  3. Ph⊕ (⊖ in LR)
35
Q

CML has a characteristic proliferation of what cell types?

A

mature myeloids esp granulocytes + basophils

36
Q

p. vera has a characteristic proliferation of what cell types?

A

mature myeloids esp RBCs***, granulocytes, plts

37
Q

p.vera is driven by ____ mutation which leads to what clinical finding?

A

JAK2 kinase mutation; hyperviscous blood

38
Q

Hypervisous blood in p.vera has what assc sx? (4)

A
  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
Q

Treat p. vera with? (3)

A
  1. phlebotomy
  2. hydroxyurea
  3. ruxolitinib
    otw death in a year
40
Q

3 ways to distinguish PV from reactive polycythemia (in PV/high altitude/ectopic renal cell carcinoma)

A
  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
Q

essential thrombocythemia (ET) is driven by ____ mutation which leads to what clinical finding?

A

JAK2 kinase mutation; ↑risk for bleeding/thrombrosis (øhyperuricemia/gout/progression to marrow fibrosis or acute leukemia)

42
Q

ET has a characteristic proliferation of what cell types?

A

mature myeloids esp plts***, granulocytes, RBCs

43
Q

Treat ET with? (3)

A

Angrelide (from TBL),
hydroxyurea,
ruxolitinib (JAK2 kinase inhibitor)

44
Q

Myelofibrosis has a characteristic proliferation of what cell types?

A

mature myeloids esp MGKs

45
Q

myelofibrosis is driven by ____ mutation which leads to what clinical finding?

A

JAK2 kinase mutation (50%); ↑PDGF causing fibrosis (↓hematopoiesis –> ↑ risk for inftn, thrombosis, and bleeding)

46
Q

Older pt presents with splenomegaly, leukoerythoblastic smear and and ↑ PDGFexpression. Dx?

Pt is at risk for (3) ?

How do you Tx?

A
  • myelofibrosis (tear drop/nucleated RBCs + immature granulocytes);
  • inftn; thrombosis, bleeding
  • tw Ruxolitinib (JAK2 inh)
47
Q

splenomegaly in myelofibrosis is dt ____

A

extramedullary hematopoeisis

48
Q

↑ 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

A

Mycosis fungoides

49
Q

in Mycosis Fungoides cells can spread to the blood, producing ______

A

Sezary Syndrome (cerebriform nucleated cells)

50
Q

↑ of mature CD+ T associated with HTLV-1. Pt presents with rash, LAD, hepatosplenomegaly, and lytic bone lesions (↑Ca+)

A

Adult T Cell Leukemia/Lymphoma

ATLL (HTLV-1common in Japan and Caribbean; ATLL ≠MM (ø AL or renal sx like CRAB)

51
Q

↑ of mature B cells positive for tartrate resistance acid phosphatase (TRAP+).

A

Hair Cell Leukemia

52
Q

Pt presents with ⊕ TRAP, dry tap on bone marrow aspiration, and splenomegaly, ⊖LAD. Dx? Tx?

A 
Hairy Cell Leukemia (dry tap = fibrosis; splenomegaly = ↑ trapped hair cells)
Tw cladribine (2CDA = adenosine deaminase inh, stops toxic acc'm)
53
Q

↑ of naive CD5 and CD20 B cells. Pt presents with generalize LAD and/or small lyphocytic lymphoma. Dx? Tx?

A

CLL

observation –> rituximab + accumPemrbrolizumab (antiPDL1)

54
Q

smudge cells are characteristic of?

55
Q

3 complications of CLL

A
  1. Hypogammaglobulineamia (death by inftn)
  2. Autoimmune hemolytic anemia
  3. Richter Transformation (to diffuse large Bcell lymphoma; enlarging lymph node or spleen)
56
Q

Crystal aggregates of _____ are seen as ______ in Acute Myeolid Leukemia (ALL)

A

myeloperoxidase (MPO); Auer Rods

57
Q

what kind of syndromes present with cytopenias, hypercellular bone marrow, abnormal maturation of cells, and increased blast (<20%)

A

myelodysplastic syndromes

58
Q

_____ is characterized by proliferation of MGKs and øMPO; also assc with down syndrome prior to 5 yo

A

Acute Megakaryoblastic leukemia (AML subtype)

59
Q

_____ is characteriszed by proliferation of monocytes and øMPO; blasts infiltrate gums

A

Acute Monocytic Leukema

60
Q

Acute Promyelocytic Leukema is driven by ____ translocation which leads to _____. Tx?

A 
t(15;17) retinoic acid rcp tranlocation --> RAR disruption + ↑ blast accumulation
tw ATRA (vitA derivative --> blast maturation)
61
Q

APL pts are at risk for what emergent complication

A

DIC (↑ primary granules)

62
Q

> 20% blasts characterizes

A

acute leukemia

63
Q

↑ acc’m of TdT⊕ lymphocytes; assoc with Down syndrome in pts under 5yo

A

Acute Lymphoblastic leukemia (T-ALL vs B-ALL)

64
Q

Teenage pt presents with thymic (mediastinal) mass and TdT⊕ lymphoblasts with ⊕CD2,3,4,7,8 and CD10⊖. Dx?

65
Q

Child pt presents with t(12;21)⊕ cytogenetic test and TdT⊕ lymphoblasts with ⊕CD10, 19, 20 and

A

B-ALL (good px)

66
Q

Elderly pt presents with t(9;22)⊕ cytogenetic test and TdT⊕ lymphoblasts with ⊕CD10, 19, 20 and

A

Ph+ ALL (B-ALL; poor px)

MS2 Unit2 (8 decks)

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