WH ALL

Which of the following ACQUIRED genetic mutation is found in T cell ALL?

• NOTCH1

• PAX-5

• ETV6-RUNX1

• TCF3-PBX1

Answer explanation

Abnormalities found in T cell ALL:

NOTCH1

HOX11L2

LYL1

TAL1

HOX11

CNS leukaemia is defined as

• Presence of at least 5 leukocytes/µL in CSF OR by presence of cranial nerve palsies

• Presence of at least 5 leukocytes/µL in CSF AND by presence of cranial nerve palsies

• Presence of at least 5 leukocytes/µL in CSF OR by presence of MRI mass lesion

• Presence of at least 5 leukocytes/µL in CSF AND by presence of MRI mass lesion

Answer explanation

CNS leukemia is defined by the presence of at least 5 leukocytes/μL of CSF (with leukemic blast cells apparent in a cytocentrifuged sample or by flow cytometry) or by the presence of cranial nerve palsies

Which of the following immunophenotype is consistent with Burkitt-type ALL?

• CD19+

  CD22+

  CD79a+

  cIg+

  sIg+

• CD19+

  CD22+

  CD79a+

  cIg+/–

  sIgμ–

  HLA-DR+

• CD2+

  CD1+/–

  CD4+/–

  CD8+/–

  HLA-DR–

  TdT+/–

• CD2–

  CD1–

  CD4–

  CD8–

  HLA-DR+/–

  TdT+

Answer explanation

19/F, with fatigue and lymphocytosis >400 x 10^9/L had new-onset fever Tmax 39C.

PE: dry lips and oral mucosa, soft abdomen, no skin lesions.

BMA showed lymphoblasts of 95%.

Pending infectious work up.

Which of the following intervention is your next best step?

• Start broad-spectrum antibiotics

• Insert IJ catheter for leukapheresis

• Give IV methylprednisolone 1 mg/kg for 3 days

• Start chemotherapy alternating HyperCVAD and MTX with cytarabine

Answer explanation

1. 1) Any patient presenting with fever, especially a patient with neutropenia, should be given broad-spectrum antibiotics until infection is excluded.

2) Leukapheresis can be used to reduce the burden of leukemic cells. But the short- and long-term benefits of the procedures are questionable

Goal of therapy in patients older than age 70 years with ALL?

• Quality of life

• MRD negativity

• Bridge to HSCT

• Reduced dose chemotherapy

Answer explanation

Cure is not common in patients older than age 70 years, maintenance of good quality of life is a major goal for this age group.

Which of the following L-asparaginase has the longest half-life?

• Calasaparaginase

• Pegasparaginase

• Erwinia L-asparaginase

• E coli L-asparaginase

Answer explanation

1) Calaspargase has a longer half-life and is administered at 2500 U/m 2 IV every 3–4 weeks

2) Pegaspargase 2500 IU/m 2 IV or intramuscularly every other week for 1–2 doses 3) Erwinia preparation, which has the shortest half-life, is administered at 20,000 IU/m 2 IV or intramuscularly three times per week for 6–12 doses.

4) E coli l-asparaginase range from 5000–10,000 IU/m 2 IV or intramuscularly administered 2–3 times per week for 6–12 doses

Which condition has beneficial outcomes in ALL?

• Autologous HCT in Ph+ ALL, MRD negative, on imatinib

• Autologous HCT in Ph-like ALL, MRD negative, on ruxolitinib

• Autologous HCT in T cell ALL, MRD negative, on nelarabine

• Autologous HCT in ALL with t(4;14), MRD negative

Answer explanation

Autologous transplantation failed to improve outcome in adult ALL overall, mainly because of a high rate of relapse.

Autologous stem cell transplantation is feasible and beneficial for adults with Philadelphia chromosome–positive ALL who achieve a molecular remission after combined chemotherapy and imatinib

Which patient requires intensive chemotherapy for treatment of Adult ALL?

• Precursor B cell with MLL gene

• Precursor B cell with ETV6-RUNX1 fusion

• Precursor B cell with hyperploidy

• Precursor B cell with leukocytosis 50,000/µL

Answer explanation

Almost all adult patients are candidates for intensive therapy. Of the many variables that influence prognosis, treatment is the most important.

Target of Blinatumumab?

• CD3+ T cells

  CD19+ ALL cells

• CD3+ T cells

  CD20+ ALL cells

• CD3+ T cells

  CD21+ ALL cells

• CD3+ T cells

  CD22+ ALL cells

Answer explanation

Blinatumomab is a bispecific T-cell engaging (BiTE) monoclonal antibody that binds both CD3+ T cells and CD19+ ALL cells.

BMA smear showed

• ALL with large blasts showing prominent nucleoli

• ALL with cytoplasmic granules

• ALL with cytoplasmic vacuolation

• ALL with fine chromatin, and indistinct nucleoli