Hematology #5 (ALL, AML, CLL, CML, HL, NHL, MM)

Question 1

What is the MC childhood hematologic malignancy?

What is the pathophysiology of this condition?

Answer 1

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

Question 2

What is pancytopenia again?

Answer 2

Low WBC’s, Low RBC’s, and Low platelets

Question 3

Symptoms of ALL

Answer 3

-Pancytopenia: Fever, infections
-Bleeding, anemia, pallor, fatigue
-Headache, stiff neck, visual changes, vomiting
-Hepatomegaly and splenomegaly MC findings
-Lymphadenopathy

Question 4

Where are the METs for ALL MC to?

Answer 4

CNS and testes

Question 5

What does a CBC with peripheral smear for ALL show?

What does bone marrow aspiration show?

Answer 5

WBC 5,000 - 100,000
Anemia
Thrombocytopenia

Hypercellular with >20% blasts (definitive)

Question 6

Treatment for ALL

Answer 6

-Combination chemotherapy

-If CNS disease: intrathecal Methotrexate

Question 7

What is the MC acute leukemia in adults?

Answer 7

Acute Myeloid Leukemia (AML)

Question 8

Explain what AML is

Answer 8

-Accumulation of leukemic blasts (immature WBC’s) in the bone marrow, peripheral blood, or occasionally other tissues. Increased production leads to pancytopenia.

Question 9

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.

Answer 9

Auer Rods

Question 10

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?

Answer 10

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

Question 11

Treatment for AML

How about for M3/APL?

Answer 11

-Combination chemotherapy

All-trans-retinoic acid can be added to M3/APL

Question 12

What is a leukostasis reaction, which is commonly seen in AML and CML?

Answer 12

Medical emergency. Leukostasis leads to increased blood viscosity and white cell plugs in the microvasculature, impeding blood flow and causing local hypoxia

Question 13

What is seen on CBC with leukostasis reaction

Answer 13

Hyperleukocytosis (WBC > 100,000) + Symptoms due to hypoxia

Question 14

Treatment for leukostasis reaction

Answer 14

-Leukapheresis

Question 15

The MC form of leukemia in adults is

Answer 15

Chronic Lymphocytic Leukemia (CLL)

Question 16

CLL is a mature B cell clonal malignancy. What are some risk factors?

Answer 16

Increasing age (70 years old is median), men

Question 17

Symptoms of CLL

Answer 17

-Asymptomatic usually
-Pancytopenia: fatigue, anemia, infections, etc.
-LAD (MC finding)
-Splenomegaly

Question 18

What is seen on CBC with peripheral smear in CLL?

Answer 18

-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)

Question 19

Treatment for CLL

Answer 19

-Radiation, stem cell transplant

Question 20

What is Chronic Myelogenous Leukemia (CML)?

Answer 20

Uncontrolled production of mature and maturing granulocytes (predominantly neutrophils)

Question 21

What is the pathophysiology of CML (it has a specific translocation and chromosome)

Answer 21

-BCR-ABL1 fusion gene

Translocation between chromosomes 9 and 22 = Philadelphia chromosome

Question 22

Symptoms of CML

Answer 22

-Splenomegaly
-Pruritus after hot showers (histamine release)

Question 23

What is seen on CBC with smear in CML?

Answer 23

-Leukocytosis with granulocytic cells (neutrophilia, basophilia, eosinophilia)

Question 24

What is one other lab that can be drawn for CML that is DECREASED and only found in functioning WBC’s, not leukemic cells?

Answer 24

Leukocyte alkaline phosphatase score (LAP)

Question 25

If the patient is Philadelphia chromosome positive, what is the treatment for CML?

Answer 25

Tyrosine kinase inhibitors (Imatinib)

Question 26

Only effective cure for CML

Answer 26

-Hematopoietic stem cell transplant

Question 27

Hodgkin's Lymphoma is a B cell malignancy. What is unique about the risk factors?

Answer 27

Bimodal: peaks at 20 then again > 50 years old RF: Epstein-Barr Virus, Smoking

Question 28

MC type of Hodgkin's Lymphoma and who is it MC in?

Answer 28

Nodular sclerosing Females MC

Question 29

Symptoms of Hodgkin's Lymphoma

Answer 29

-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

Question 30

What is true about Systemic (B) Symptoms?

Answer 30

They indicate advanced disease

Question 31

What is done to diagnose Hodgkin's Lymphoma?

Answer 31

Excision whole lymph node biopsy: Reed-Sternberg Cell (large cells with multi lobed nuclei) owl-eye appearance and inclusions in nuclei

Question 32

Treatment for Hodgkin's Lymphoma

Answer 32

-Early: Chemo + Radiation -Advanced: Chemo is mainstay --ABVD: Adriamycin, Bleomycin, Vinblastine, Dacarbazine

Question 33

What is the MC type of Non-Hodgkin Lymphoma? Explain it

Answer 33

-Diffuse large B cell: Fast growing, aggressive form (rapidly enlarging lymph nodes)

Question 34

What is a Burkitt Lymphoma?

Answer 34

Type of NHL associated with EBV infection. MC seen in adolescent/pediatric and HIV patients in Africa. -Involves the jaw and facial bones.

Question 35

Symptoms of NHL

Answer 35

-Painless LAD -GI tract MC site of extranodal involvement -Fever, night sweats, weight loss

Question 36

What is required for diagnosis and classification of NHL?

Answer 36

Lymph node and/or tissue biopsy

Question 37

What is multiple myeloma (Plasmacytoma)?

Answer 37

Cancer associated with proliferation of a single clone of plasma cells, leading to increased production of ineffective monoclonal antibodies (IgG and IgA, IgM)

Question 38

Multiple Myeloma is the MC primary bone malignancy in adults. What are some risk factors? Explain the pathophysiology behind this condition.

Answer 38

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.

Question 39

Symptoms of Multiple Myeloma (BREAK your bones)

Answer 39

Bone pain: Vertebral involvement MC, ribs due to osteolytic lesions Recurrent Infections Elevated calcium Anemia Kidney Injury: increased BUN and Cr

Question 40

What is seen on the following diagnostics in Multiple Myeloma? Serum Protein Electrophoresis: Urine protein electrophoresis: CBC: Skull Radiographs: Bone Marrow Aspiration:

Answer 40

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%

Question 41

What is the definitive diagnostic for MM?

Answer 41

Bone marrow aspiration

Question 42

Treatment for Multiple Myeloma

Answer 42

-Autologous stem cell transplant most effective therapy then chemotherapy