PANCE Blueprint Hematology (5%)

Patient with acute lymphocytic leukemia (ALL) will present as → a 5 y/o child with bone pain, bleeding, and fever. His mom notes that the patient has been less active and has had recurrent upper respiratory infections over the past several months. Clinical examination reveals diffuse lymphadenopathy. His CBC demonstrates anemia and leukopenia, and the blood smear shows peripheral blasts. Bone marrow biopsy demonstrates 30% lymphoblasts.

Patient with chronic lymphocytic leukemia (CLL) will present as → a 60-year-old male complains of fatigue. On physical exam, he has painless cervical lymphadenopathy and hepatosplenomegaly. His blood tests demonstrate severe anemia, decreased neutrophil count, and immature B cells that broke apart during the peripheral smear.

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Acute lymphocytic leukemia (ALL): CHILD + lymphadenopathy + bone pain + bleeding + fever in a CHILD, bone marrow > 20% blasts in bone marrow

• In acute leukemia, there’s uncontrolled proliferation of partially developed WBCs (blast cells), which build up in the blood over a brief period of time
• ALL results from a proliferation of cells from the lymphoid line (vs. the myeloid line in AML) and is classified into T-cell ALL, where there’s proliferation of T-cell precursors, and B-cell ALL (70-80% of cases), where there’s proliferation of B-cell precursors
• Caused by chromosomal translocations (12,21) and (9,22)
• Population: Children - most common childhood malignancy peak age 3-7
• Symptoms of ALL include fatigue because of the anemia, easier bleeding (gingival bleeding, bruising, and nosebleeds) because of thrombocytopenia, and more frequent infections because of the leukopenia
• Abnormal lymphoblasts in ALL can also infiltrate the lymph nodes and other lymphatic tissue, so it’s also called acute lymphoblastic lymphoma
• A key clinical symptom in T-cell ALL is that the abnormal lymphoblasts migrate to the thymus like normal T-cells do and cause thymus enlargement, which can present as a mass or growth in the mediastinum.
• Highly responsive to chemotherapy (remission > 90%)

Chronic Lymphocytic Leukemia (CLL): Middle age/elderly patient, often asymptomatic (seen on blood tests), fatigue, lymphadenopathy, and splenomegaly

• Population: Adults - peak age 50 y/o
• It is not caused by one particular mutation but results from a variety of chromosomal mutations that affect lymphocytes, in particular B cells
• Just like with the blast cells in ALL, partially mature B-cells can move into the lymphatic tissues and cause lymphadenopathy. B cells in these nodes will accumulate to form “lymphomas,” which is why CLL is sometimes called small lymphocytic lymphoma
• These abnormal B Cells are likely to produce autoantibodies, resulting in immune thrombocytopenia and autoimmune hemolytic anemia
• Hairy cell leukemia is a special but rare type of CLL linked with a mutation in the BRAF gene. The affected B-cells have hair-like projections, giving them a fuzzy appearance
• Up to 70% of persons with CLL are asymptomatic at diagnosis, and when symptoms do appear, they are typically nonspecific and include easy fatigability and weight loss. Some patients can present with lymphadenopathy and hepatosplenomegaly
• Diagnostic studies: SMUDGE CELLS on peripheral smear, mature lymphocytes
• Treatment with observation: if lymphocytes are > 100,000 or symptomatic, treat with chemo

ALL

• CBC: pancytopenia (anemia, thrombocytopenia, leukopenia)
• Peripheral blood smear demonstrates peripheral blasts. Blast cells in the peripheral smear may approach 90% of the white blood cell (WBC) count
• Bone marrow aspiration and biopsy: with > 20% blasts
• Histochemical studies, cytogenetics, and immunophenotyping studies help distinguish the blasts of ALL from those of AML or other disease processes

Several lymphoblasts in the peripheral blood of a patient with acute lymphoblastic leukemia

CLL

• CBC: pancytopenia (anemia, thrombocytopenia, leukopenia) with an increase in the absolute number (>5,000) of lymphocytes in the peripheral blood
• The peripheral blood smear of patients with CLL demonstrates lymphocytosis (small mature lymphocytes) and often shows “smudge” cells, which are immature B cells that have broken apart during the smear
• Evaluation of the bone marrow is not usually necessary

Smudge cell in a peripheral blood smear

Acute Lymphoblastic Leukemia (ALL): Chemotherapy

• Highly responsive to combination chemotherapy with a remission of > 90%.
• Stem cell transplant if relapse.

Chronic Lymphoblastic Leukemia (CLL): Observation if indolent

• Chronic - treat with chemo