E2 PATHO WEEK 7 (10.13)

Overview of White Blood Cell Disorders

  • Focuses on specific disorders related to white blood cells.

  • General theme: Disorders of white blood cells can involve either overproduction or underproduction of cells, similar to red blood cell disorders.

White Blood Cells and Their Function

  • White blood cells (WBCs) are crucial components of the immune system, responsible for fighting infections.

  • Lifespan:

    • Red blood cells: Approximately 120 days.

    • White blood cells: Approximately 10 days.

  • Composition of Blood Cells:

    • WBCs constitute about 1% of the total blood cell volume.

Types of White Blood Cells

  • White blood cells are divided into two main categories:

    • Granulocytes: Include neutrophils, eosinophils, and basophils.

    • Agranulocytes: Include lymphocytes (B cells, T cells) and monocytes.

Disorders Relating to White Blood Cells

  • Disorders generally fall into two broad categories:

    • Proliferative disorders (overproduction of WBCs)

    • Deficient disorders (underproduction of WBCs)

Underproduction of White Blood Cells
  • Neutropenia: A condition characterized by low neutrophil counts, which can cause increased susceptibility to infections.

  • Thrombocytopenia: A condition of decreased platelet count.

  • Agranulocytosis: The absence of neutrophils; effectively, a severely low or nearly zero count.

    • Prefixed ‘A’ indicates absence.

Causes of Neutropenia
  1. Accelerated removal: Increased destruction of neutrophils, possibly due to rapid apoptosis.

  2. Drug-induced granulocytopenia: Certain medications, such as Clozaril (an antipsychotic), can induce low neutrophil counts.

  3. Cyclic neutropenia: Commonly seen in children, characterized by fluctuating neutrophil counts.

  4. Felty syndrome: A combination disorder associated with rheumatoid arthritis that includes neutropenia and splenomegaly (enlarged spleen).

Mononucleosis

  • Caused by Epstein Barr Virus (EBV).

  • Commonly known as the 'kissing disease' due to its transmission through saliva.

  • Characteristics of Mononucleosis:

    • Once infected, EBV stays dormant in B lymphocytes for life.

    • Self-limiting illness: The illness resolves without requiring extensive medical intervention, usually within one to two weeks.

  • Symptoms:

    • Enlarged lymph nodes (especially in axilla and groin).

    • Fever, malaise, chills.

Lymphomas

Hodgkin's Lymphoma vs. Non-Hodgkin's Lymphoma

  • Hodgkin's Lymphoma:

    • Characterized by the presence of Reed-Sternberg cells.

    • Swollen lymph nodes, rarely extranodal (staying within lymph nodes).

    • Better prognosis due to limited spread; mainly affects younger adults.

  • Non-Hodgkin's Lymphoma:

    • No Reed-Sternberg cells present.

    • Can be extranodal (spreads beyond lymph nodes).

    • Generally worse prognosis because it often metastasizes.

Ann Arbor Staging for Lymphomas
  • Utilizes four stages to classify severity based on the number of lymph nodes involved:

    • Stage I: Involvement of a single lymph node.

    • Stage II: Involvement of two lymph nodes on the same side of the diaphragm.

    • Stage III: Involvement of lymph nodes on both sides of the diaphragm.

    • Stage IV: Dissemination of the disease to extralymphatic sites.

Leukemias

Classification of Leukemias

  • Four main types:

    1. Acute Lymphocytic Leukemia (ALL)

    2. Chronic Lymphocytic Leukemia (CLL)

    3. Acute Myelogenous Leukemia (AML)

    4. Chronic Myelogenous Leukemia (CML)

Acute vs. Chronic Leukemias
  • Acute: Involves immature cells; often serious and fast-growing.

  • Chronic: Involves mature cells; generally slower progression.

  • Cell Origin:

    • Myeloid (from bone marrow) or lymphoid (from lymphatic tissues).

Terminology

  • Terminal plasma cells: These are mature plasma cells that cannot further divide, and their malignancy indicates multiple myeloma, characterized by bone pain and reabsorption.

  • M protein: A biomarker used in diagnosing multiple myeloma; its presence indicates the disease.

Key Points to Remember
  • When dealing with leukemias, focus on the type of cells involved (mature or immature) and their origin (myeloid or lymphoid).

  • For Hodgkin's disease, confirm the presence or absence of Reed-Sternberg cells.

Important Statistics and Values

  • Normal erythrocyte sedimentation rate for men: 0 to 15 mm/hr.

  • Hemoglobin levels generally range, with values being critical in assessing health (e.g., not less than 11 g/dL for females).