Hematology #5 (ALL, AML, CLL, CML, HL, NHL, MM) Flashcards
What is the MC childhood hematologic malignancy?
What is the pathophysiology of this condition?
Acute Lymphocytic Leukemia (ALL)
occurs in 2-5 years of age = ALL my children
Overpopulation of immature WBC’s (blasts) overtake normal hematopoiesis, resulting in pancytopenia
What is pancytopenia again?
Low WBC’s, Low RBC’s, and Low platelets
Symptoms of ALL
-Pancytopenia: Fever, infections
-Bleeding, anemia, pallor, fatigue
-Headache, stiff neck, visual changes, vomiting
-Hepatomegaly and splenomegaly MC findings
-Lymphadenopathy
Where are the METs for ALL MC to?
CNS and testes
What does a CBC with peripheral smear for ALL show?
What does bone marrow aspiration show?
WBC 5,000 - 100,000
Anemia
Thrombocytopenia
Hypercellular with >20% blasts (definitive)
Treatment for ALL
-Combination chemotherapy
-If CNS disease: intrathecal Methotrexate
What is the MC acute leukemia in adults?
Acute Myeloid Leukemia (AML)
Explain what AML is
-Accumulation of leukemic blasts (immature WBC’s) in the bone marrow, peripheral blood, or occasionally other tissues. Increased production leads to pancytopenia.
There are three types of AML. One type, acute promyelocytic leukemia (APL or M3), is associated with DIC and is noted with the presence of __________ and myeloperoxidase positivity.
Auer Rods
Pancytopenia symptoms are symptoms of AML including general fatigue, dyspnea, weakness, mucocutaneous bleeding, fever, infections.
What is the best initial test for AML?
What is the GOLD standard and what is seen on this test?
Initial: CBC with peripheral smear shows normocytic normochromic anemia
GOLD: Bone marrow biopsy shows >20% myeloblasts and Auer Rods (pink/red rod-like granular structures in cytoplasm) in APL
Treatment for AML
How about for M3/APL?
-Combination chemotherapy
All-trans-retinoic acid can be added to M3/APL
What is a leukostasis reaction, which is commonly seen in AML and CML?
Medical emergency. Leukostasis leads to increased blood viscosity and white cell plugs in the microvasculature, impeding blood flow and causing local hypoxia
What is seen on CBC with leukostasis reaction
Hyperleukocytosis (WBC > 100,000) + Symptoms due to hypoxia
Treatment for leukostasis reaction
-Leukapheresis
The MC form of leukemia in adults is
Chronic Lymphocytic Leukemia (CLL)
CLL is a mature B cell clonal malignancy. What are some risk factors?
Increasing age (70 years old is median), men
Symptoms of CLL
-Asymptomatic usually
-Pancytopenia: fatigue, anemia, infections, etc.
-LAD (MC finding)
-Splenomegaly
What is seen on CBC with peripheral smear in CLL?
-Absolute, isolated lymphocytosis ( WBC >20,000)
–Small, well-differentiated normal appearing lymphocytes with smudge-cells (lab artifact when fragile B cells crushed by cover slip during preparation)
Treatment for CLL
-Radiation, stem cell transplant
What is Chronic Myelogenous Leukemia (CML)?
Uncontrolled production of mature and maturing granulocytes (predominantly neutrophils)
What is the pathophysiology of CML (it has a specific translocation and chromosome)
-BCR-ABL1 fusion gene
Translocation between chromosomes 9 and 22 = Philadelphia chromosome
Symptoms of CML
-Splenomegaly
-Pruritus after hot showers (histamine release)
What is seen on CBC with smear in CML?
-Leukocytosis with granulocytic cells (neutrophilia, basophilia, eosinophilia)
What is one other lab that can be drawn for CML that is DECREASED and only found in functioning WBC’s, not leukemic cells?
Leukocyte alkaline phosphatase score (LAP)
If the patient is Philadelphia chromosome positive, what is the treatment for CML?
Tyrosine kinase inhibitors (Imatinib)
Only effective cure for CML
-Hematopoietic stem cell transplant
Hodgkin’s Lymphoma is a B cell malignancy. What is unique about the risk factors?
Bimodal: peaks at 20 then again > 50 years old
RF: Epstein-Barr Virus, Smoking
MC type of Hodgkin’s Lymphoma and who is it MC in?
Nodular sclerosing
Females MC
Symptoms of Hodgkin’s Lymphoma
-Asymptomatic painless LAD**
-Lymph node pain after alcohol ingestion
-Mediastinal LAD or mass
-Hepatomegaly, Splenomegaly
-Neck (Cervical and Supraclavicular) LAD
-B Symptoms: night sweats, weight loss, cyclical fever
What is true about Systemic (B) Symptoms?
They indicate advanced disease
What is done to diagnose Hodgkin’s Lymphoma?
Excision whole lymph node biopsy: Reed-Sternberg Cell (large cells with multi lobed nuclei) owl-eye appearance and inclusions in nuclei
Treatment for Hodgkin’s Lymphoma
-Early: Chemo + Radiation
-Advanced: Chemo is mainstay
–ABVD: Adriamycin, Bleomycin, Vinblastine, Dacarbazine
What is the MC type of Non-Hodgkin Lymphoma?
Explain it
-Diffuse large B cell: Fast growing, aggressive form (rapidly enlarging lymph nodes)
What is a Burkitt Lymphoma?
Type of NHL associated with EBV infection. MC seen in adolescent/pediatric and HIV patients in Africa.
-Involves the jaw and facial bones.
Symptoms of NHL
-Painless LAD
-GI tract MC site of extranodal involvement
-Fever, night sweats, weight loss
What is required for diagnosis and classification of NHL?
Lymph node and/or tissue biopsy
What is multiple myeloma (Plasmacytoma)?
Cancer associated with proliferation of a single clone of plasma cells, leading to increased production of ineffective monoclonal antibodies (IgG and IgA, IgM)
Multiple Myeloma is the MC primary bone malignancy in adults. What are some risk factors?
Explain the pathophysiology behind this condition.
Elderly > 65, African Americans, Men, Benzene Exposure
Plasma cells accumulate in bone marrow, interrupting bone marrow’s normal cell production. Protein accumulation causes kidney injury.
Symptoms of Multiple Myeloma (BREAK your bones)
Bone pain: Vertebral involvement MC, ribs due to osteolytic lesions
Recurrent Infections
Elevated calcium
Anemia
Kidney Injury: increased BUN and Cr
What is seen on the following diagnostics in Multiple Myeloma?
Serum Protein Electrophoresis:
Urine protein electrophoresis:
CBC:
Skull Radiographs:
Bone Marrow Aspiration:
Serum Protein: Monoclonal protein spike (IgG)
Urine Protein: Bence-Jones Protein (kappa or lambda chains)
CBC: Rouleaux formation (RBC’s with a stack of coins appearance). Increased ESR.
Skull Radiographs: punched out lytic lesions.
Bone Marrow Aspiration: plasmacytosis > 10%
What is the definitive diagnostic for MM?
Bone marrow aspiration
Treatment for Multiple Myeloma
-Autologous stem cell transplant most effective therapy then chemotherapy
