PANCE Blueprint Hematology (5%)

Acute and chronic lymphocytic leukemia (Lecture)

Patient with acute lymphocytic leukemia (ALL) will present as → a 3-7 y/o child with lymphadenopathy, bone pain, bleeding, and fever. Bone marrow demonstrates > 20% lymphoblasts

Patient with chronic lymphocytic leukemia (CLL) will present as →60-year-old male complains of fatigue, blood tests demonstrate severe anemia, decreased neutrophil count, and small, abnormal B lymphocytes in the bone marrow (>30%) with levels at 90,000 per cubic millimeter. Has painless cervical lymphadenopathy and hepatosplenomegaly.

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

  • Population: Children - most common childhood malignancy peak age 3-7
  • Highly responsive to chemotherapy (remission > 90%)

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

  • Population: Adults - most common form of leukemia in adults - peak age 50 y/o
  • Diagnostic studies: SMUDGE CELLS on peripheral smear, mature lymphocytes
  • Treatment with observation, if lymphocytes are > 100,000 or symptomatic treat with chemo

ALL - Bone marrow with > 20% blasts

CLL - diagnosed with peripheral smear shows fragile B-cells that smudge during slide prep "smudge cells"

Smudge cells are fragile B cells that often smudge during slide preparation

Smudge cells are fragile B cells that often smudge during slide preparation - seen in CLL

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
IM_Acute_Lymphoblastic_Leukemia_ASSETS_ Acute lymphoblastic leukemia (ALL) is a neoplasm of immature B and T cells (pre B and pre T cells), which are referred to as lymphoblasts. About 85% of ALLs are B cell tumors and typically manifest in children less than 15 years of age. The less common T cell ALLs tend to present in adolescents as a thymic lymphoma in the mediastinum. In these cancers, the bone marrow is typically hypercellular and packed with lymphoblasts, which replace the normal elements within the bone marrow. Therefore, common symptoms include fatigue due to anemia and bleeding due to thrombocytopenia. Neoplastic infiltration of bone marrow can also cause bone pain from marrow expansion and infiltration of the subperiosteum. Immunostaining for terminal deoxynucleotidyl transferase (TdT), a specialized DNA polymerase that is expressed only in pre B and pre T lymphoblasts is positive in more than 95% of cases. Majority of these B-cell tumors also express the common acute lymphoblastic leukemia antigen (CALLA). Approximately 90% of ALLs demonstrate chromosomal changes, most commonly hyperploidy of chromosomes. These chromosomal aberrations are thought to cause the dysregulation of the expression and function of transcription factors that are required for normal B and T cell development. A fraction of B cell tumors contain a translocation of genes 12;21 involving the genes TEL and AML1, two genes required in very early hematopoiesis. This translocation is associated with better prognosis. This tumor is known to spread to the CNS and testes. With aggressive chemotherapy, about 95% of children with ALL obtain complete remission.

Acute Lymphoblastic Leukemia (ALL) Picmonic

chronic-lymphocytic-leukemia-cll_5045_1466637131 Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the United States. Most patients are older than 60 years of age and there is a 2 to 1 male predominance. In CLL, lymph nodes are diffusely effaced by an infiltrate of small lymphocytes. The peripheral blood also contains large numbers of small round lymphocytes with minimal cytoplasm. Some of these cells often come disrupted in the process of making smears and produce smudge cells that are characteristic of CLL. The bone marrow is almost always involved. Unlike most other lymphoid malignancies, chromosomal translocations are relatively rare. Patients are often 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. Hypogammaglobulinemia is also common and contributes to disruption of normal immune function. CLL is also associated with warm autoimmune hemolytic anemia, which is often caused by the attachment of IgG antibodies to red blood cells.

Chronic Lymphocytic Leukemia (CLL) Picmonic

Question 1
A 60-year-old presents with fatigue and splenomegaly. CBC reveals the following: WBC- 24,000/microliter, Hgb- 13.5 g/dl, Hct- 40%, MCV- 87 fL, MCHC- 34 g/dl, MCH- 28 pg, and platelets- 380,000/mL. The differential reveals neutrophils- 11%, lymphocytes- 80%, monocytes- 8%, and basophils- 1%. What is the most likely diagnosis?
A
Acute lymphocytic leukemia
Hint:
Acute lymphocytic leukemia is more common in children and presents with blasts in the peripheral bloodAcute lymphocytic leukemia is more common in children and presents with blasts in the peripheral blood
B
Acute myelogenous leukemia
Hint:
Acute myelogenous leukemia presents with pancytopenia and presence of blasts in the peripheral blood.
C
Chronic lymphocytic leukemia
D
Chronic myelogenous leukemia
Hint:
Chronic myelogenous leukemia presents with elevated WBC count, marked left shift in the myeloid series of cells, and positive for Philadelphia chromosome.
Question 1 Explanation: 
Chronic lymphocytic leukemia presents with a WBC count greater than 20,000/microliter and absolute lymphocyte count of greater than 5000/microliter.
Question 2
A 3-year-old girl presents for her well-child visit. The physician notes pallor and bruising. On physical examination, the vital signs are normal, but there is a generalized lymphadenopathy and hepatosplenomegaly. A complete blood count (CBC) reveals a white blood cell (WBC) count of 33,000/ mm3 and a platelet count of 81,000/ mm3. The peripheral blood smear shows blasts, and the lactate dehydrogenase activity is elevated. CBC shows blasts as well as hypochromic red blood cells. The most common leukemia among children is which of the following?
A
acute myelogenous leukemia (AML)
Hint:
AML is the most common leukemia in adults, but it is less common in children.
B
acute lymphocytic leukemia (ALL)
C
chronic myelogenous leukemia (CML)
Hint:
CML is very rare in children, occurring in approximately 2% of childhood leukemia cases.
D
chronic lymphocytic leukemia (CLL)
Hint:
CLL is also unusual in children.
E
equal numbers of AML and ALL
Question 2 Explanation: 
Seventy-three percent of childhood leukemia is ALL. AML is the most common leukemia in adults, but it is less common in children. CML is very rare in children, occurring in approximately 2% of childhood leukemia cases. CLL is also unusual in children.
Question 3
Most cases of ALL are diagnosed between which of the following age ranges?
A
birth and 2 years
Hint:
See B for explanation
B
2 and 3 years
C
12 and 15 years
Hint:
See B for explanation
D
8 and 12 years
Hint:
See B for explanation
E
5 and 15 years
Hint:
See B for explanation
Question 3 Explanation: 
The peak incidence for ALL is between 2 and 3 years of age.
Question 4
Which ethnic group has the highest frequency of ALL?  
A
white
Hint:
The frequency for whites is 30 to 40 per 1 million,
B
Hispanic
C
African American
Hint:
African Americans is approximately one-third the rate of whites.
D
Asian
E
no ethnic variation
Question 4 Explanation: 
Hispanics have a frequency of 43 per 1 million. The frequency for whites is 30 to 40 per 1 million, and that for African Americans is approximately one-third the rate of whites.
Question 5
Which of the statements regarding ALL is true?
A
the peak age at onset is 12 years
Hint:
The peak incidence for ALL is between 2 and 3 years of age.
B
at the time of diagnosis, most patients have a thrombocytosis
Hint:
Most patients with ALL will have a decreased hemoglobin level and platelet count at the time of diagnosis
C
CBC with differential is the most useful initial test
D
a chest radiograph is the most useful initial test
Hint:
A chest radiograph should be obtained to search for a possible mediastinal mass.
Question 5 Explanation: 
The most useful initial test to perform in patients suspected of having ALL is a CBC with differential. In most cases, there will be an increase or decrease in at least one or two cell types. The WBC count may be decreased, normal, or elevated, and the differential will show a neutropenia. Blasts may be seen on the peripheral smear. Most patients with ALL will have a decreased hemoglobin level and platelet count at the time of diagnosis. The diagnosis of ALL is confirmed by a bone marrow examination showing more than 25% lymphoblasts. A chest radiograph should be obtained to search for a possible mediastinal mass. With combination chemotherapy, the prognosis of ALL is very good.
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