1. LEUKEMIA
CASE STUDY 2

2. The patient was a 40-year-old white woman admitted to the Duke University Medical Center in May 1984 with an eight– month history of progressive fatigue and a three-month history of gradually worsening generalized weakness, exercise intolerance, anorexia, and a 20-pound weight loss.

3. A submandibular mass was noted six months prior to admission, which slowly increased in size until the time of her presentation. On to Duke Hospital, her physical examination revealed diffuse adenopathy in cervical, supraclavicular, submandibular, axillary, and inguinal regions, along with hepatosplenomegaly.

4. Her initial peripheral blood examination revealed:
Hemoglobin of 7.1 g/dl
Hematocrit of 21.5%
Platelet count of 31,000/mm3
white blood cell count of 35,000/mm3 with 95% blasts.

5. The blasts were large, with a high nuclear-cytoplasmic ratio and prominent nucleoli. A bone marrow aspirate and biobsy demonstrated near complete pereplacement by large vacuolated blasts with intensely basophilic cytoplasm.

6. Special stains of the marrow blasts revealed:
Cytogentic studies of the marrow blasts t(8;22) and t(14;18) Translocation.
The tumor cells in strongly express markers of B cell differentiation (CD20, CD22, CD19), as well as CD10 and BCL6
PAS +
Myeloperoxidase -
Suddan black
Non-specific esterase stains

7. Laboratory Finding:
Normal range
gm/dL
140, ,000 cells/mcL
/mm3 %
Hb=7.1 g/dl 31,000/mm3 =Plt count 35,000/mm3 =WBCs 21.5% =HCT Blast cell = 95% Cytochemical Reactions: MPO Negative SBB Negative NSE Negative PAS Positive

8. Acute Lymphoblastic Leukemia
Discussion
The most likely diagnosis is ALL-L3
Acute Lymphoblastic Leukemia
lymphoblastic leukemia (also called ALL or acute lymphocytic leukemia) is a cancer of the blood and bone marrow.
This type of cancer usually gets worse quickly if it is not treated.
In a patient with ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. The cells do not work like normal lymphocytes and are not able to fight infection very well. These cells are cancer (leukemia) cells. Also, as the number of leukemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may lead to infection, anemia, and easy bleeding.

10. ALL-L3 This is the rarest form of ALL.
The cell size is large, with fine, homogenous nuclear chromatin containing prominent nucleoli. The The nucleus is regular oval to round.
The cytoplasm is moderately abundant and is deeply basophilic and vacuolated.

11. Signs and symptoms of ALL include the following:
Fever
Decreased neutrophil count
Signs and symptoms of anemia, such as pallor, fatigue, dizziness, palpitations, cardiac flow murmur, and dyspnea with even mild exertion
Bleeding (eg, from thrombocytopenia due to marrow replacement)
Disseminated intravascular coagulation (DIC) at diagnosis (about 10% of cases)
Palpable lymphadenopathy
Symptoms related to a large mediastinal mass (eg, shortness of breath), particularly with T-cell ALL
Bone pain (severe and often atypical)
Left upper quadrant fullness and early satiety due to splenomegaly (about 10-20% of cases)
Symptoms of leukostasis (eg, respiratory distress, altered mental status)
Renal failure in patients with a high tumor burden
Infections, including pneumonia
Petechiae (particularly on lower extremities) and ecchymoses
Signs relating to organ infiltration with leukemic cells and lymphadenopathy

12. Diagnosis
Laboratory tests and other studies used in the workup for ALL include the following:
Complete blood count with differential.
Coagulation studies.
Peripheral blood smear.
Chemistry profile, including lactic dehydrogenase, uric acid, liver function studies, and bun/creatinine.
Appropriate cultures (in particular, blood cultures) in patients with fever or other signs of infection.
Chest x-ray.
Computed tomography.
Multiple-gated acquisition scanning.
Electrocardiography.
Bone marrow aspiration and biopsy (definitive for confirming leukemia).
Immunohistochemistry.
Flow cytometry.
Cytogenetics.
Polymerase chain reaction.
Gene expression profiling.

13. The main types of treatment used for ALL are:
Chemotherapy
Targeted therapy
Stem cell transplant
Other treatments such as surgery, radiation therapy, or monoclonal antibodies, may be used in special circumstances.
Treatment of ALL typically lasts for about 2 years. It is often intense, especially in the first few months of treatment, so it is important that you are treated in a center that has experience with this disease