Objectives

1. Define Hodgkin’s Lymphoma, Non-Hodgkin’s Lymphoma, Leukemias & Multiple Myeloma.
2. Discuss the pathophysiology of all disease processes.
3. Describe clinical manifestations of all disease processes.
4. Discuss diagnostic and staging procedures.
5. Describe treatment options and modalities for disease processes

Introduction to Hematological Neoplasms

📌Cellular regulation:The genetic and physiologic processes that control cellular growth, replication, differentiation, and function of maintaing homeostasis.

Common hematological Neoplams.

1. Leukemia
2. Lymphoma
3. Multiple Myeloma.

✨Hematopoietic malignancies are often classified by the cells involved; Myeloid cells include monocytes, macrophages, neutrophils, basophils, eosinophils, erythrocytes, and megakaryocytes to platelets. Lymphoid cells include T cells, B cells, natural killer cells, and innate lymphoid cells.

Leukemia

♦️Leukemia is a malignant disorder of the bone marrow characterized by a disturbance in the early development of hemopoietic factors especially WBCs with over 20% of blasts in the marrow. Absence of orderly differentiation & maturation in a specific cell line.

♦️The defect originates in the hematopoietic stem cell, the myeloid, or the lymphoid stem cell line. The unregulated proliferation of leukocytes i.e leukemic cells in the bone marrow results in crowding & leaves little room for the production of normal cells.

♦️Leukemic cells are released into the bloodstream, infiltrate & are ineffective in the inflammatory process.

Classification of Leukemia

▶️Leukemia can be classified on the basis of ;a) according to the stem cell line involved

1. Myeloid
2. Lymphoid

📌Myeloid cells include monocytes, macrophages, neutrophils, basophils, eosinophils, erythrocytes, and megakaryocytes to platelets. Lymphoid cells include T cells, B cells, natural killer cells, and innate lymphoid cells.

b).Also classified as either

1. Acute Leukemia
2. Chronic Leukemia

Acute Leukemia

✍🏼The onset of symptoms is abrupt.

✍🏼The leukocyte development halted as blast phase.

✍🏼The progresses rapidly with aggressive treatment.

Chronic Leukemia

✍🏼The symptoms evolve over months to years.

✍🏼The majority of leukocytes are mature.

✍🏼Progresses slower, and may extend for years.

Leukemia Risk Factors.

1.  Exposure to radiation or chemotherapy
2. Congenital disorders-Down syndrome, Fanconi anemia , neurofibromatosis
3. Genetic mutations- result in loss of normal cell function.
4.  Viral infections.

Acute Myeloid Leukemia.(AML)

♦️It is most common form of leukemia, affecting 90% of disease in adults. It affects any age group with the average age at diagnosis is 68 years.

 Risk Factors

•  Increased age
•  Male
• Chemical exposure: benzene, pesticides
• Ionizing radiation
• Prior chemotherapy treatments
• Smoking
• Genetic disorders: Down’s syndrome

Clinical Manifestations

•  Neutropenia: fever, infection.
• Anemia: pallor, fatigue, weakness, dyspnea on exertion, dizziness, increased heart rate and decreased BP.
•  Increased WBC and increased blood viscosity: increased BP with bounding pulse.
• Thrombocytopenia: ecchymosis, petechiae, epistaxis, gingival bleeding.
• Organs: pain due to enlargement of liver or spleen.
• Lymphadenopathy.
• Anxiety.

Diagnostics

• Peripheral blood smear- may be enough to diagnosis.
• WBC may be high, low or normal, absolute neutrophil count (ANC) low.
• High WBC consisting of mostly blast cells has a poorer prognosis.
• Decreased platelets, RBC’s, decreased hemoglobin & hematocrit.
• Blood clotting factors- decreased fibrinogen level and increased PTT.
• Bone marrow aspirate & smear- Definitive test includes proteins or antigenic markers that provide information on the type of leukemia present.
•  Lumbar puncture- test CSF for leukemic cells.
• Increased uric acid levels
•  Increased LDH
• Chest x-ray
• Evaluation of myocardial function- MUGA scan, echocardiogram.

Medical Management

📌Induction chemotherapy-designed to clear the bone marrow of overt disease- usually 4 weeks

♦️Induce a complete remission – bone marrow with less than 5% lymphoblasts and no leukemic cells in peripheral blood, normal platelet counts, neutrophils above 1500

♦️Primary agents- Cytarabine, Anthracycline and corticosteroids – 72- 92% remission rate may add targeted therapy using midostaurin or gemtuzumab ozogamicin.

♦️Allopurinol 300mg 1-3 x per day for resolution of hyperuricemia.

✨Induction chemotherapy-CNS may also have to be treated- with intrathecal chemo with drugs that cross blood-brain barrier-Methotrexate will be used to treat if there are leukemic cells in CNS and/or prophylactically.

📌Consolidation or Intensification therapy- One must be in remission to begin this phase. It solidifies remission response & kills any remaining leukemic cells. The phase may last 1-3 months, consist of another course of the drugs used for induction in different doses or a totally different combination of chemo drugs.

📌Maintenance therapy- involves use of low-dose medication for 2-3 years.

📌Bone marrow transplant- used in high-risk clients. Allogeneic transplantation may be used as part of intensification therapy, it usually Increases risk of long-term survival.

📌Monoclonal antibodies are often combined with standard chemo or as single agent.

🕹️Occurance of relapse-one should start on inducation therapy again.