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)

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

=>Myeloma

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 (LA-PV)

• 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

Translocation- in AML and CML
Transformation in CLL

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
    FRC

elderly- measure FRC

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 Adriamycin), Oncovin, Prednisolone.

Burkitt’s lymphoma- Childhood tumor, fast growing, highly responsive to chemo- risk of tumor lysis.- tt with combination of chemo and immunotherapy.

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%