Leukemia Overview and Types

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Questions and Answers

What is the primary use of chemotherapy in treating leukemia?

To specifically target benign tumors

To prevent blood cell production

To cure and control systemic diseases (correct)

To enhance the immune response

What constitutes the definition of acute leukemia according to WHO?

Complete absence of normal blood cells

Presence of leukocytosis with no other symptoms

Less than 10% bone marrow blasts

More than 20% bone marrow blasts (correct)

What is the main characteristic of leukemia?

Development of non-cancerous blood cells

Increased effectiveness of normal cell function

Reduction in bone marrow size

Over-proliferation of malignant cells (correct)

What does leukocytosis indicate when it becomes chronic?

Possible presence of malignancy Signup and view all the answers

What is the obsolete classification system frequently taught in academic settings for acute leukemia?

FAB classification Signup and view all the answers

What information does the WHO classification for leukemia utilize beyond cellular morphology?

Immunologic probes of cell markers Signup and view all the answers

Which type of leukemia is most commonly associated with anemia?

Under myelogenous leukemia Signup and view all the answers

What is a key characteristic of leukemic cells in the bone marrow?

Replacement of normal bone marrow elements Signup and view all the answers

What is a common risk associated with a decrease in neutrophil count?

Increased risk of infection Signup and view all the answers

Which type of leukemia is characterized by the rapid expansion of immature cells?

Acute Lymphocytic Leukemia Signup and view all the answers

What is the typical clinical onset feature difference between acute and chronic leukemia?

Sudden onset in acute leukemia Signup and view all the answers

What complication is associated with thrombocytopenia in leukemia patients?

Increased risk of bleeding Signup and view all the answers

How does chronic leukemia affect other organs?

Hypertrophy and fibrosis due to cell infiltration Signup and view all the answers

What is a typical time frame for the untreated progression of chronic leukemia?

2-6 years Signup and view all the answers

What types of cells predominantly characterize chronic leukemia?

Mature cells Signup and view all the answers

What physical condition may arise from hypermetabolism in leukemia patients?

Muscle atrophy Signup and view all the answers

Which cell type is a hallmark of Classic Hodgkin Lymphoma?

Reed-Sternberg cells Signup and view all the answers

What is the common characteristic of Nodular Lymphocyte Predominant Hodgkin Lymphoma?

Presence of Popcorn cells Signup and view all the answers

How is Acute Leukemia generally characterized?

Sudden onset and rapid progression Signup and view all the answers

Which test is typically used to confirm the diagnosis of Classic Hodgkin Lymphoma?

Immunophenotyping Signup and view all the answers

What distinguishes Chronic Leukemia from Acute Leukemia?

Presence of mature cells Signup and view all the answers

What are the common presentations in Non-Hodgkin Lymphoma when Reed-Sternberg cells are absent?

Enlarged lymph nodes Signup and view all the answers

Which leukemia has the presence of smudge cells in the peripheral blood?

Chronic Lymphocytic Leukemia Signup and view all the answers

What is a distinguishing feature of Acute Myelogenous Leukemia (AML)?

Unregulated proliferation of Myeloid cells Signup and view all the answers

What percentage of blasts is required for the classification of Acute myeloblastic leukemia M1?

<blockquote> 20% </blockquote> Signup and view all the answers

Which characteristic is NOT associated with Acute myeloblastic leukemia M2?

No granules Signup and view all the answers

What does the presence of >5% abnormal eosinophils indicate in the context of M4eo classification?

Acute myelomonocytic leukemia with eosinophilia Signup and view all the answers

Which of the following esterase tests is positive in Acute erythroleukemia M6?

Nonspecific Esterase Signup and view all the answers

Auer rods are commonly associated with which type of leukemia?

Acute monoblastic leukemia M5 Signup and view all the answers

In which classification is the presence of Megakaryoblasts and cytoplasmic budding stated?

M7 Signup and view all the answers

Which classification demonstrates >20% monoblasts?

M4 Signup and view all the answers

What is indicated by the PAS test in Acute monoblastic leukemia M5?

Positive Signup and view all the answers

Which classification does NOT typically demonstrate any granules?

M0 Signup and view all the answers

What morphological feature is characteristic of Acute myeloblastic leukemia M1?

Auer rods absent Signup and view all the answers

What genetic abnormality is primarily affected in Chronic Myelogenous Leukemia?

BCR-ABL gene Signup and view all the answers

What condition is characterized by increased blood volume and viscosity?

Polycythemia Vera Signup and view all the answers

Which progenitor cell is primarily affected in Polycythemia Vera?

Common Myeloid Progenitor (CMP) Signup and view all the answers

What is the WBC count threshold that indicates leukocytosis in Chronic Myelogenous Leukemia?

<blockquote> 300 x 10^9/L </blockquote> Signup and view all the answers

What diagnostic test is commonly used to confirm Chronic Myelogenous Leukemia?

FISH (Fluorescence In Situ Hybridization) Signup and view all the answers

Which of the following conditions results in apparent increases in RBC level but is not true polycythemia?

Pseudopolycythemia Signup and view all the answers

Which syndrome is associated with a relative rise in red blood cell level due to stress?

Gaisbock Syndrome Signup and view all the answers

In Polycythemia Vera, the abnormal condition arises from what source?

Abnormal bone marrow stem cells Signup and view all the answers

Study Notes

Leukemia

Leukemia is a group of malignant diseases of hematopoietic tissue.
It is characterized by the replacement of normal bone marrow elements with malignant cells.
Leukemia is known as blood cancer.
Leukemia is characterized by the over-proliferation of malignant cells.
The presence of many malignant cells is found in both bone marrow and circulation.
Leukocytosis is a normal response to infection.
It may indicate malignancy when it becomes chronic, progressively elevates, or occurs without an obvious cause.

Types of Leukemia

Acute Leukemia:
- Characterized by the rapid progression of abnormal expansion of immature cells or blasts.
- Has a sudden onset and rapid progression.
- Blood smear shows blast cells or immature cells.
Chronic Leukemia:
- Characterized by the slow progression of abnormal expansion of mature cells.
- Has a gradual onset and slow progression.
- Mature cells are over-proliferated in bone marrow and circulation.

Classification

Fab Classification:
- Based on cellular morphology and cytochemical stains.
- Defines acute leukemia as greater than 20% bone marrow blasts.
- Commonly used in academic teaching.
- Is obsolete but based on the principles of WHO Classification.
WHO (World Health Organization) Classification:
- Classification based on:
  - Cellular morphology
  - Cytochemical stains
  - Immunologic probes of cell markers
  - Cytogenetics
  - Molecular abnormalities
  - Clinical syndrome
- Defines acute leukemia as greater than 20% bone marrow blasts.
- Uses cell markers, such as cluster of differentiation (CD), which are WBC specific.
- Anchored on the principle of FAB classification, using cellular morphology and cytochemical stains.
- More specific because it detects cell markers, clinical syndrome, and genetic defects.

Clinical Manifestations of Leukemia

Effects of Bone Marrow Suppression:
- Anemia: Decreased production of red blood cells, especially if the type of leukemia is under myelogenous.
- Infection risk: Decreased neutrophil count, which is critical for fighting infections.
- Bleeding risk: Thrombocytopenia leads to decreased clotting factors.
- Physiological fracture risk: Proliferation of cells in the bone marrow weakens the periosteum.
Effects of Infiltration of Malignant Cells:
- Hypertrophy and fibrosis occur in organs like the liver, spleen, and lymph glands.
- Increased intracranial pressure, ventricular dilation, and meningeal irritation
Hypermetabolic State:
- Cells are deprived of nutrients.
- Causes loss of appetite, weight loss, general fatigue, and muscle atrophy.

Lymphoblastic Leukemia

Acute Lymphoblastic Leukemia (ALL):
- Sudden onset with rapid progression.
- Makes up around 95% of cases of Hodgkin lymphoma in developed countries.
- Hallmarks:
  - Presence of Reed-Sternberg cells, the cancer cells.
  - Owl's eye appearance.
- Tests include: CBC, PBS, skin biopsy, immunophenotyping, TCR gene rearrangement test, and flow cytometry.
- CD markers: CD2, CD3, and CD4 Positive.
Myelocytic/Myelogenous Leukemia:
- Acute Myelogenous Leukemia (AML):
  - Sudden onset with rapid progression.
  - Presence of blast cells in peripheral blood and bone marrow.
  - Classified as L1, L2, and L3.
- Chronic Myelogenous Leukemia (CML):
  - Gradual onset with slow progression.
  - Presence of mature cells in the peripheral blood and bone marrow.
  - Appearance of smudge cells in the peripheral blood smear.

Non-Hodgkin Lymphoma

Classic Hodgkin Lymphoma (cHL):
- Accounts for about 95% of all cases of Hodgkin lymphomas in developed countries.
- Hallmark: Reed-Sternberg cells.
- Appearance of enlarged lymph nodes may be the first sign.
Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL):
- Usually starts in lymph nodes of the neck or armpit.
- More common in men than women.
- Treatment differs from classic types.
- Features:
  - Popcorn cells, variant of Reed-Sternberg cells, are present.
  - Absence of Reed-Sternberg cells.

Chronic Myelogenous Leukemia (CML)

Diagnosis:
- WBC count greater than 300 x 10^9/L (Leukocytosis).
- Positive FISH (Fluorescence In Situ Hybridization).
- Bone marrow aspirate to assess the bone marrow cell environment.
Pathogenesis:
- Affects the BCR-ABL gene, leading to the Philadelphia chromosome.
- Affects the Common Myeloid Progenitor (CMP), disrupting cell production.

Polycythemia Vera

Increased blood volume and viscosity, leading to hyperviscosity syndrome.
Disrupts the production of cells by affecting the Common Myeloid Progenitor (CMP).
Types:
- Pseudopolycythemia: Apparent rise in RBC level in the blood due to factors like Burns, Dehydration, and Stress.
- True Polycythemia: Chronic condition resulting from abnormal bone marrow stem cells.
  - Primary: True Polycythemia.
  - Secondary: Caused by conditions like Gaisbock Syndrome.

FAB Classification Summary

MPO: Myeloperoxidase
SBB: Sudan Black B
PAS: Periodic Acid Schiff

Name or Disorder	Morphology	MPO	SBB	Specific Esterase	Nonspecific Esterase	PAS
M0: Acute myeloblastic leukemia: minimally differentiated	>20% blasts, No granules	–	–	–	–	–
M1: Acute myeloblastic leukemia with no maturation	>20% blasts, Few granules +/- Auer rods	+	+	–	–	–
M2: Acute myeloblastic leukemia with promyelocytic leukemia	>20% blasts, 20% promyelocytes, Prominent granules, ++ Auer rods, Faggot cells	++	++	+	–	–
M4: Acute myelomonocytic leukemia	>20% blasts, >20% monoblasts, + Auer rods	±	±	±	++	–
M4eo: Acute myelomonocytic leukemia with eosinophilia	>20% blasts, >20% monocytes, >5% abn eos, + Auer rods	±	±	±	++	–
M5: Acute monoblastic leukemia with or without maturation	>20% blasts, >80% monocytes and promonocytes with/without differentiation	±	±	–	++	++
M6: Acute erythroleukemia	>80% erythroid, >30% proerythroblast, + Auer rods	–	–	+	+++	+
M7: Acute megakaryocytic leukemia	>30%, >50% megakaryoblasts, Cytoplasmic budding	+	–	+	+++	+

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Description

This quiz covers the fundamental aspects of leukemia, including its definition, characteristics, and the difference between acute and chronic types. Understand the progression and symptoms associated with each form of leukemia. Test your knowledge on this critical topic in hematology.