Heme-Oncology

Hemochromatosis

• Etiology:
  • -autosomal recessive disorder
    
  • -excessive iron absorption
    
  • -causing increased levels of iron
    
  • -swollen liver
    
  • -bronze skin color
    
  • -could develop diabetes

Polycythemia Vera (PV)

• Etiology:
  • Myeloproliferative Disorder
    
  • -abnormal uncontrolled proliferation of RBCs
    
  • -JAK2 mutation

Sickle Cell Disease (anemia)

• Etiology:
• • -inherited hemolytic
    
  • -autosomal recessive causes abnormality in hemoglobin synthesis
    
  • -RBC are sickle-shaped

Platelet deficiency

• Presentation:
  • hematuria, petechiae, bruising, heavy menstrual flow, gum bleeding

WBC deficiency

• Presentation:
  • fatigue, lymphadenopathy, frequent infections, weight loss

RBC deficiency

• Presentation:
  • delayed capillary, refill, pale mucus membranes, fatigue and pale skin

Leukemia

• Etiology:
  • malignant disorder of bone marrow and usually blood
    
  • uncontrolled production of leukocytes
    
  • pancytopenia if cells crowd bone marrow

Acute leukemias

• Etiology:
• • presence of immature cells, usually blast cells

Chronic leukemias

• Etiology:
  • mature, malfunctioning cells

Acute Lymphocytic Leukemia (ALL)

• Etiology:
  • -too many lymphoblasts
    
  • -most common in children

Acute Myelogenous Leukemia (AML)

• Etiology:
  • -too many myeloblasts
    
  • -most common ADULT leukemia

Chronic Lymphocytic Leukemia (CLL)

• Etiology:
  • -too many immature lymphocytes
    
  • -most common ADULT leukemia in Western world
    
  • -B lymphocytes

Chronic Myelogenous Leukemia (CML)

• Etiology:
  • -too many blood cells made in bone marrow
    
  • -PHILADELPHIA CHROMOSOME
    
  • -mostly in adults

Multiple Myeloma (MM)

• Etiology:
  • -BENCE JONES PROTEIN
    
  • -infiltrate bone marrow and aggregate into tumor masses in skeletal system
    
  • -M proteins (become most prominent protein in blood)
    
  • -malignant proliferation of plasma cells
    
  • -B cell cancer
    
  • -hypercalcemia, renal failure, bone lesions

Malignant lymphomas

• Etiology:
  • -diverse group of neoplasms that develop from proliferation of malignant lymphocytes in the lymphoid system
    
  • -REAL classification
    
  • -Hodgkin/Non-Hodgkin

Hodgkin Lymphoma

• Etiology:
  • -presence of REED-STERNBERG cells
    
  • -S&S = fever, weight loss, night sweats, pruritus
    
  • -Can usually be cured w/ early diagnosis & treatment

Non-Hodgkin Lymphoma (NHL)

• Etiology:
  • -linked to chromosomal translocations
    
  • -Risk factors = male, older, white, certain immune disorders, certain environmental exposures, viruses, diet high in fats and meats

Burkitt Lymphoma (Non-Hodgkin)

• Etiology:
  • -Epstein-Barr virus (EBV)
    
  • -translocation of chromosome 8
    
  • -very fast growing tumor of the jaw and facial bones
    
  • -most common type of non-Hodgkin lymphoma in children

Infectious Mononucleosis (IM)

• Etiology:
  • -caused by Epstein-Barr Virus (EBV)
    
  • -acute, self-limiting infection of B lymphocytes
    
  • -Testing = monospot
    
  • -Serious complications = splenic rupture ( could cause death)

Disseminated Intravascular Coagulation (DIC)

• Etiology:
  • -complex, acquired disorder in which clotting and hemorrhage simultaneously occur.
    
  • -sepsis is the most common condition associated with this.
    
  • -D- dimer and elevated FDP

Heparin-Induced thrombocytopenia (HIT)

• Etiology:
  • – IgG antibodies target heparin-platelet factor complex and activates platelet aggregation and more clots form.

Immune (Idiopathic) thrombocytopenia purpura (ITP)

• Etiology:
  • – decreased platelets
    
  • -Hypersensitivity II reaction (IgG autoimmune antibody)
    
  • -acute form often develops secondary to viral infection
    
  • -petechiae & purpura –> hemorrhage

Hemophilia

• Etiology:
  • -inherited disease (sex related recessive)
    
  • -causes insufficient production of clotting factors

Von willebrand disease

• Etiology:
  • -inherited autosomal dominant
    
  • -deficiency of von willebrand factor (vwf) a protein that carried clotting factor in blood.

Thrombotic thrombocytopenic purpura (TTP)

• Etiology:
  • -platelets aggregate, form microthrombi and cause occlusion of arteries and capillaries
    
  • -plasma atom TS13 defect

Vitamin K Deficiency

• Etiology:
  • Vit K is necessary for synthesis and regulation of prothrombin, the procoagulant factors and proteins C & S (anticoagulants)
    
  • -This causes the inability to produce a clot