ASH Image Bank (2001); doi:10.1182/ashimagebank-2001-100187
Copyright © 2001 by the American Society of Hematology.
Precursor T-Lymphoblastic Leukemia/Lymphoma
John Lazarchick, M.D.
Medical University of South Carolina
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Figure 2. Blasts with basophilic, agranular cytoplasm, irregular, folded nuclear borders, and containing one or more nucleoli. These cells were MPO and dual esterase negative. Single myelocyte is present.
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Figure 3. Blast population expressing dim CD45 is present (A). These cells were CD34-positive (not shown) and constituted 75% of all marrow cells analyzed.
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Figure 4. Blast population identified by CD45 versus sidescatter is CD7- and CD13-positive. Surface CD3 was negative (not shown).
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Figure 5. Total replacement of marrow by monomorphic blast population. Normal hematopoietic elements can not be identified.
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Figure 8. T-cell beta receptor gene rearrangement is present on all patient (Pt) restriction enzyme digests. C = + and - controls.
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Figure 9. Normal [46 XY(7cells)] and near tetraploid [92 XXYY(13 cells)] metaphases were present. This hyperdiploid karyotype is not specific for myeloid or lymphoid malignancy.
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Clinical Summary
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A 25-year-old African American man was presented to the emergency department because of hemoptysis. He complained of a 2-week history of fatigue and myalgias. He was on no medications and his past medical history was non-contributory.
Physican examination: Afebrile. Head,eyes, ears, nose, and throat (HEENT) - petechiae on buccal mucosa and tonsillar enlargement. Lungs clear to auscultation. Cervical adenopathy - multiple shotty nodes bilaterally. Axillary adenopathy, right greater than left. Spleen tip palpable 2 cm below the left costal margin. Neurologic exam was normal.
Lab: Hemoglobin (Hgb) 6.5g/dl, white blood cells (WBC) 6500 (15P, 5Bands, 24L, 1M, 55 blasts), platelets 51,000/ul; lactate dehydrogenase (LDH) 2547IU/L; albumin 2.7g/dL, globulin 2.0g/dL; cerebro spinal fluid (CSF) - normal; HIV - negative; chest x-ray - small bilateral effusion; CT scans -cervical, axillary adenopathy, no anterior mediastinal mass.
Sex
Male
Age
25
Ethnicity
African American
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Diagnosis
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The presence of blasts in the peripheral blood, with associated anemia and thrombocytopenia, and generalized adenopathy limit the differential diagnosis to acute leukemia. The major differential issue is of assignment of blast lineage so that appropriate therapy can be given.
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Discussion
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Acute lymphoblastic leukemia (ALL) represents approximately 12% of all leukemias and occurs in a bimodal pattern, most occurring in children with a second smaller peak in the elderly. ALL of T-cell lineage represents approximately 20% to 30% of all ALL cases diagnosed with the majority being of B-cell lineage. Within the T-cell lineage group, most are of thymic origin (12% of children with ALL; 18% of adults with ALL) and a small percentage are prethymic (bone marrow) in origin (1% of all children with ALL; 6% of all adults with ALL). PreThymic T-cell ALL has a unique immunophenotype in that these cells are characteristically CD7 positive but lack other T-cell lineage markers. In contrast, T-cell ALL of thymic origin are characteristically CD7-, CD2-, CD3-, CD4- or CD8-positive depending on the degree of maturation of the malignant clone within the thymus. The reliability of immunophenotypic analysis alone to determine lineage can be misleading since 5% to 20% of de novo cases of AML can be CD7 positive, especially in cases of AML-M0 and M1.Conversely, CD13 and CD33 can be seen in approximately 10% of cases of ALL. When faced with such diagnostic pitfalls, it is necessary to assess for the presence of cytoplasmic CD3 (cCD3), the most specific marker of T-cell lineage. This is not routinely performed on flow cytometric analysis because it requires that the cells be permeabilized prior to analysis, but can be readily performed on marrow biopsy material using immunohistochemical staining. With the positive results noted in this case, it would not have been necessary to perform TdT staining or Southern blot analysis but these can be useful in the rare case where cCD3 is absent. The therapeutic implications of assigning correct lineage are evident.
The clinical presentation and laboratory findings in PreThymic T-cell acute lymphoblastic leukemia/lymphoma are well illustrated in this case. Fatigue is secondary to the degree of anemia. Petechiae and ecchymoses are seen secondary to the thrombocytopenia and infrequently due to disseminated intravascular coagulation (DIC). Peripheral adenopathy may be massive in T-cell ALL but is not as marked in prethymic T-ALL. Similarly, an anterior mediastinal mass (thymus) is common in T-ALL but is uncommon to rare in prethymic T-ALL.
Hyperleukocytosis with WBCs greater than 100,000/ul is a feature of T-ALL but normal- to mildly-elevated counts are characteristic of preT-ALL.
Elevated LDH, a reflection of tumor burden and turnover, was present in this case and is a common finding in lymphoid malignancies. The utility of cytogenetic analysis as a tumor marker and prognostic indicator in cases of acute leukemia should also be noted. Although chromosomal abnormalities, both in number (hyper- and hypodiploidy) or structure (translocations) are relatively common in ALL, the near tetraploidy (96XXYY) noted in this case is seen only in 1% of all cases of ALL and preferentially in preT-ALL.
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Differential Diagnosis
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The presence of blasts in the peripheral blood, with associated anemia and thrombocytopenia and generalized adenopathy limit the differential diagnosis to acute leukemia. The major differential issue is of assignment of blast lineage so that appropriate therapy can be given.
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Chapter 11: Acute lymphoblastic leukemia and lymphoblastic lymphoma
Copyright © 2001 by the American Society of Hematology.
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Clinical Summary
Diagnosis
