Hematological Malignancies and Blood Cancer Types

Hematological Malignancies

• Cancer is uncontrolled cell growth with loss of apoptosis (programmed cell death), occurring in any cell in the body.

Types of Hematological Malignancies

• Leukemia: originates from myeloid or lymphoid cells.
• Lymphoma: originates from lymphoid tissue (in bone marrow or peripheral).
• Myeloma: originates from plasma cells (a type of B cell).

Review of Marrow Components

• Neutrophils: can develop into Acute Myeloid Leukemia.
• Lymphocytes: can develop into Acute Lymphocytic Leukemia.
• Monocytes: can develop into Acute Myeloid Leukemia.
• Eosinophils: can develop into Hypereosinophilic Syndrome.
• Basophils: can develop into AML, seen in CML, and also in HD.
• RBC's: can develop into Acute Erythroid Leukemia (rare).
• Platelets: can develop into Acute Megakaryoblastic Leukemia (rare).

Normal Maturation of a Neutrophil

• Myeloblast → Promyelocyte → Myelocyte → Metamyelocyte → Band →Neutrophil.

Epidemiology of Acute Leukemias

• Generally affects older people, non-Hispanic whites.
• Risk factors include genetic disorders (e.g., Down Syndrome, Ataxia telangiectasia, Fanconi Anemia, Li Fraumeni), radiation exposure, and chemical exposure.

Epidemiology of Chronic Leukemias

• Only known risk factor is exposure to ionizing radiation.
• CML was the first disorder proven to be caused by a genetic translocation [t(9;22)].

Epidemiology of Lymphomas

• Relatives of probands have a 3.5x higher chance of developing lymphoma.
• Higher incidence in patients with autoimmune diseases (e.g., RA, hemolytic anemia, psoriasis, SLE, Systemic sclerosis, Polyarteritis nodosa, sarcoidosis), immunosuppression, and viral infections (HIV, HTLV, EBV).

Epidemiology of Multiple Myeloma

• Affects multiple parts of the body, hence the name.
• Higher incidence in African Americans and those with a higher body mass index.
• Risk increases with exposure to agent orange (especially those with MGUS).
• Higher incidence in families (3.5x higher with 1st-degree relative).

Myelodysplastic Syndrome (MDS)

• Not a malignancy, but a pre-malignancy of the bone marrow.
• Defined as a disorder in which the bone marrow does not produce enough healthy blood cells.

Hematological Malignancies

• Cancer is uncontrolled cell growth with loss of apoptosis (programmed cell death), occurring in any cell in the body.

Types of Hematological Malignancies

• Leukemia: originates from myeloid or lymphoid cells.
• Lymphoma: originates from lymphoid tissue (in bone marrow or peripheral).
• Myeloma: originates from plasma cells (a type of B cell).

Review of Marrow Components

• Neutrophils: can develop into Acute Myeloid Leukemia.
• Lymphocytes: can develop into Acute Lymphocytic Leukemia.
• Monocytes: can develop into Acute Myeloid Leukemia.
• Eosinophils: can develop into Hypereosinophilic Syndrome.
• Basophils: can develop into AML, seen in CML, and also in HD.
• RBC's: can develop into Acute Erythroid Leukemia (rare).
• Platelets: can develop into Acute Megakaryoblastic Leukemia (rare).

Normal Maturation of a Neutrophil

• Myeloblast → Promyelocyte → Myelocyte → Metamyelocyte → Band →Neutrophil.

Epidemiology of Acute Leukemias

• Generally affects older people, non-Hispanic whites.
• Risk factors include genetic disorders (e.g., Down Syndrome, Ataxia telangiectasia, Fanconi Anemia, Li Fraumeni), radiation exposure, and chemical exposure.

Epidemiology of Chronic Leukemias

• Only known risk factor is exposure to ionizing radiation.
• CML was the first disorder proven to be caused by a genetic translocation [t(9;22)].

Epidemiology of Lymphomas

• Relatives of probands have a 3.5x higher chance of developing lymphoma.
• Higher incidence in patients with autoimmune diseases (e.g., RA, hemolytic anemia, psoriasis, SLE, Systemic sclerosis, Polyarteritis nodosa, sarcoidosis), immunosuppression, and viral infections (HIV, HTLV, EBV).

Epidemiology of Multiple Myeloma

• Affects multiple parts of the body, hence the name.
• Higher incidence in African Americans and those with a higher body mass index.
• Risk increases with exposure to agent orange (especially those with MGUS).
• Higher incidence in families (3.5x higher with 1st-degree relative).

Myelodysplastic Syndrome (MDS)

• Not a malignancy, but a pre-malignancy of the bone marrow.
• Defined as a disorder in which the bone marrow does not produce enough healthy blood cells.

Heme Malignancies

• Leukemias:
  • Myeloid:
    • Acute: AML (sudden, >20% blasts, severe)
    • Chronic: CML (Philadelphia chromosome)
  • Lymphoid:
    • Acute: ALL (kids)
    • Chronic: CLL (older, smudge cells)
• Lymphomas:
  • Hodgkin's:
    • Peak at young age
    • Peak at old age
    • "Owl eyes"
    • Reed-Sternberg cells
  • Non-Hodgkin's:
    • All ages and races
    • Splenomegaly
    • Implants
    • H. pylori
• Multiple Myeloma:
  • Mild back pain
  • Mid-thoracic back pain
  • Not alleviated when lying down
  • Middle age
  • Pain when in supine position
  • Can cause cord suppression
• MDS (Myelodysplastic syndrome):
  • Pre-cancer but will be cancer if left untreated
  • Premalignant disorder

Hospice and Palliative Care

• Origins:
  • Cultural shift in perspectives on dying (Kubler-Ross)
  • Technological and medical advances
  • Dying is not simply a medical event
  • It is a process, an experience, that is physical, social, mental, emotional, and spiritual
• CORE Promises:
  • Your story is unique
  • Your voice matters
  • Your story will be honored
  • Stories unfold and evolve
  • Understanding comes from knowing
• Hospice:
  • Provides a philosophy of care and tools
  • Supports loved ones after death
  • 1/3 of patients die within 7 days of being in hospice
  • Challenges a death-denying culture
  • Changes the medical mindset from "nothing more we can do" to "there is much to be done"
• The hospice team:
  • A circle of care offering expertise and compassion
  • Plan of care is established and constantly updated
  • Provides medical equipment, supplies, medications, and 24-hour on-call
  • Offers bereavement care, physical therapy, nutritionist, and more

Advanced Illness Care Continuum

• Palliative Care:
  • Focuses on quality of life for anyone dealing with serious illness
  • May be just one consultation or comprehensive care
  • Focuses on establishing goals of care, relieving suffering, and navigating complex medical decisions
• Eligibility:
  • Any diagnosis
  • Any age
  • Any time in the course of disease
  • Any patient with uncontrolled symptoms
• Not all palliative care is the same:
  • Program variations
  • Team make-up
  • Services provided
  • Location of services provided

Oncology

• Definition of Cancer:
  • Unregulated cell growth
  • Abnormal tissue that grows more rapidly than normal
  • Can be solid or liquid (hematologic)
• Common Cancers:
  • Top 10 cancers by rates of new cases
  • Top 10 cancers by rates of cancer death
  • Increased death in men compared to women
  • Health care disparities and lack of access = increased risk of cancer
• Risk Factors:
  • Non-modifiable: genetic predisposition, sex, age
  • Modifiable: tobacco, nutrition, physical activity, radiation
  • Vaping and electronic cigarette aerosol
  • Tobacco cessation is the most important treatment for preventing cancer
• Staging:
  • Refers to where a cancer is located and if it has spread
  • Determines treatment options and prognosis
  • TNM system:
    • T: tumor location, size, and level of invasion
    • N: absence or presence and extent of nodal metastases
    • M: absence or presence of systemic metastases
• Treatment:
  • Surgery
  • Chemotherapy
  • Radiation Therapy
  • Oncology care:
    • Physicians/Surgeons/Radiologists
    • PAs/NPs
    • Nurse Educators
    • Chemotherapy Nurses
    • Pharmacists
    • Social Workers
    • Counselors/Psychologists
    • Financial Support Experts
  • Survivorship

Oncologic Emergencies

• Definition: common oncologic emergency condition
• Classifications:
  • Mechanical/Obstructive
  • Metabolic or Hormonal
  • Treatment-Related
• Examples:
  • Superior Vena Cava (SVC) Syndrome
  • Spinal Cord Compression
  • Hypercalcemia
  • Febrile Neutropenia
  • Tumor Lysis Syndrome
  • Anemia
  • Pulmonary Embolism
  • Thrombocytopenia
  • Urinary Obstruction
  • SIADH
  • Bleeding Disorders
  • Malignant Effusions
  • Clotting disorders### Paraneoplastic Syndromes
• Paraneoplastic syndromes refer to features of a cancer that cannot be attributed to its direct invasive or metastatic properties
• Symptoms are due to aberrant tumor production of hormones, peptides, or cytokines or to the body's immune attack on normal tissues due to shared antigens with tumor tissues
• Small cell lung cancer (SCLC) is the one type of cancer most likely to be associated with paraneoplastic syndromes

Superior Vena Cava Syndrome

• Superior vena cava syndrome (SVCS) is the clinical manifestation of superior vena cava (SVC) obstruction
• SVC obstruction results in severe reduction in venous return from the head, neck, and upper extremities
• Malignant tumors (lung cancer, lymphoma, metastatic tumors) are responsible for the majority of SVCS cases
• Symptoms include neck and facial swelling, dyspnea, and cough
• Characteristic physical findings are dilated neck veins, an increased number of collateral veins covering the anterior chest wall, cyanosis, and edema of the face, arms, and chest

Diagnosis and Treatment of Superior Vena Cava Syndrome

• Diagnosis of SVCS is a clinical one
• First step is to order a chest radiograph (CXR)
• CXR finding: widening of the superior mediastinum, most commonly on the right side
• CT provides the most reliable view of the mediastinal anatomy
• Radiation therapy is the primary treatment for SVCS caused by non-small-cell lung cancer and other metastatic solid tumors
• Chemotherapy is effective when the underlying cancer is small-cell carcinoma of the lung, lymphoma, or germ cell tumor

Spinal Cord Compression

• Malignant spinal cord compression (MSCC) is defined as compression of the spinal cord and/or cauda equina by an extradural tumor mass
• Thoracic spine is the most common site (70%), lumbosacral spine (20%), cervical spine (10%)
• Lung cancer is the most common cause
• Symptoms: localized back pain and tenderness, radicular pain, Lhermitte's sign
• Treatment: radiation therapy plus glucocorticoids, neurosurgery in some cases

Hypercalcemia

• Hypercalcemia: elevated levels of calcium in the blood (>10.5 mg/dL)
• Affects 20–30% of patients with cancer at some point during their illness
• Most common cancers causing hypercalcemia are myeloma, breast carcinoma, and NSCLC
• Symptoms: anorexia, nausea, fatigue, constipation, and polyuria
• Diagnostic workup: calcium level corrected for albumin, ionized calcium level
• Treatment: IV 0.9% saline for rehydration, bisphosphonate for moderate or severe hypercalcemia, control of tumor mass

Febrile Neutropenia

• Febrile neutropenia refers to the clinical presentation of fever and
• Cytotoxic chemotherapeutic agents affect bone marrow function
• Complications of myelosuppression result from the predictable sequelae of the missing cells' function