Haematological Malignancy- Classification

Question 1

Classification of Haem Malignancies

Answer 1

*Leukemias:

*	Acute: ALL (Acute Lymphoblastic), 
             AML (Acute Myeloid)
*	Chronic: CLL (Chronic Lymphocytic)
                   CML (Chronic Myeloid)

*	Hodgkin
*	Non-Hodgkin (NHL):
      -	High-grade vs Low-grade

Question 2

Acute Lymphoblastic Leukemia (ALL)
Q. Cells involved
Q. age
Q. Special feature
Q. Treatment

Answer 2

• Disorder of lymphoid precursor cells
  
  • Most common in childhood
  • CNS involvement common → may need craniospinal irradiation
  • Treatment:
    *Induction: 4-5 drug regimen:
    Prednisone, Vincristine, L- Asparaginase, Anthracyclines (PVLA)
    *Consolidation: Chemo or HCT
    *Maintenance: Methotx or 6MP
    Methotrexate for CNS prophylaxis
  • Good prognosis

Question 3

Acute Myeloid Leukemia (AML)
Q. Cells involved
Q. age
Q. Special feature
Q. Treatment

Answer 3

• More common in elderly
  
  • Can evolve from myelodysplasia (MDS)
  • Subtypes:
  • M3 (APML):
    -t(15;17) translocation
  • Responds well to ATRA
  • Auer rods in blasts(cytoplasmic inclusion bodies which contain Myeloperoxidase enzyme that stains blue)
  • promyelocytes release thromboplastin-Risk of DIC
  • Overall poor prognosis

Question 4

Other AML Categories

Answer 4

AML with multilineage dysplasia
AML with mixed/ambiguous lineage
Therapy-related AML (post chemo/radiation)
AML not otherwise classified

Question 5

Chronic Lymphocytic Leukemia (CLL)
Q. Age group affected
Q. Symptoms
Q.Special feature
Q. Treatment

Answer 5

• Can be classified as leukemia or low-grade lymphoma
  
  • Mostly affects elderly
  • Symptoms: fatigue, night sweats, lymphadenopathy, Painful splenomegaly, autoimmune haemolytic anemia
  • Median survival: 8–12 years
  • Richter’s transformation → DLBCL (poor prognosis)-median survival <1yr
  • Treatment: Fludarabine, Cyclophosphamide, Rituximab
    (Ritu’s Cyclone Flu)

Leukemia primarily begins in the marrow- and affects WBCs
Lymphoma primarily affects lymphocytes

Question 6

Chronic Myeloid Leukemia (CML)
Q. Translocation
Q. special Feature
Q.Treatment

Answer 6

• t(9;22) → Philadelphia chromosome–>
  
  • BCR-ABL fusion gene → Tyrosine kinase activity—->Uncontrolled proliferation of mature + immature myeloid cells
  • Treatment: Imatinib (TKI)

Question 7

Lymphomas
Q. Types and subtypes
Q Symptoms
Q.Treatment

Answer 7

**Hodgkin:

*	Young patients (20s–30s)
*	B symptoms: fever, night sweats, weight loss,pruritus, Lymphadenopathy
*	Characteristic: Reed-Sternberg cells (Giant cells of B cell lineage)
*	Treated with chemo (ABVD) ± radiotherapy(for bulky tumors)

**# Non-Hodgkin:

*	Low-grade: indolent, slow-growing
*	High-grade: aggressive, chemotherapy responsive

*	Subtypes:  Low grade-
*	Mantle cell lymphoma
*	Waldenström’s macroglobulinemia
*Follicular lymphoma

High Grade:
*DLBCL- m/c in middle age/elderly)
* Burkitt’s lymphoma (fast-growing in children)

ABVD:
Adriamycin(Doxorubicin), Bleomycin, Vinblastine, Dacarbazine
RCHOP:
Rituximab, Cyclophosphamide, Doxorubicin(aka Hydroxydaunorubicin or Doxorubicin or Adriamycin), Oncovin, Prednisolone.

Burkitt’s lymphoma- Childhood tumor, fast growing, highly responsive to chemo- risk of tumor lysis.

Question 8

Diffuse Large B-Cell Lymphoma (DLBCL)

Answer 8

• Most common NHL in elderly
  
  • Treated with R-CHOP:
  • Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone
  • Potentially curable

Question 9

Multiple Myeloma

Answer 9

• Accounts for 10% of haematological malignancies
  #Symptoms:
  
  • Bone pain, fractures
  • Bone marrow failure
  • Renal failure (due to light chain cast nephropathy or amyloidosis)
  • Hypercalcemia (from bone resorption)
  • Not curable, but chemo induces remission
  • High-dose chemo + stem cell transplant prolongs survival
  • 5-year survival ~35%