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Acute Leukemias - Republican Scientific Medical Library

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112 Chapter 7 · <strong>Acute</strong> Lymphoblastic Leukemia: Clinical Presentation, Diagnosis, and Classification<br />

Fig. 7.1. Varied cytomorphology of lymphoblasts in comparison to<br />

Burkitt leukemia cells and hematogones (Wright-stained blood and<br />

bone marrow aspirate smears). (a) Small uniform blasts, previously<br />

called “L1” type, are about two times the size of erythrocytes, and<br />

have a smudgy homogenous chromatin without prominent nucleoli.<br />

Comparison to small lymphocyte (right) is always helpful. (b) Varied<br />

lymphoblasts, including numerous larger blasts with more open<br />

chromatin, prominent nucleoli and abundant cytoplasm (previously<br />

considered “L-2 “ type). The presence of a few small “L1” blasts in the<br />

background is always helpful in considering ALL. (c) Burkitt leukemia<br />

cells (previously called “L3” blasts) are usually distinctive with<br />

homogeneous large size, and deep blue cytoplasm with prominent<br />

“L1” and “L2” blasts, recognized in Wright-stained material,<br />

is also usually not possible. Burkitt leukemia<br />

does, however, have a particular histologic pattern.<br />

The features are similar to the lymph node involvement<br />

by Burkitt lymphoma. These features are illustrated in<br />

Fig. 7.2.<br />

Hypocellular presentations of ALL are relatively<br />

rare, but can present a diagnostic challenge due to the<br />

paucity of cells and limited material for immunophenotyping<br />

[20]. Some cases of ALL can present with frank<br />

fibrosis [21], but increased reticulin is more common.<br />

Some cases are inaspirable due to the fibrosis or to<br />

the dense packing of the marrow by lymphoblasts. Necrosis<br />

is present in a small number of cases and can<br />

complicate the diagnosis, due to the lack of viable cells<br />

for either morphologic evaluation or for immunophe-<br />

vacuoles. Vacuoles can, however, be seen in some cases of AML and<br />

ALL. (d) Some lymphoblasts can be small with more clumped<br />

chromatin, and can be difficult to distinguish morphologically from<br />

CLL cells. (e) Granular lymphoblast (arrow). These blasts may resemble<br />

myeloblasts, but the granules are myeloperoxidase negative.<br />

(f) Lymphoblast with nuclear cleft, (g) So-called “hand-mirror” cells<br />

are sometimes an artifact of poor preparations, as they are not<br />

equally distributed on the slide. (h) Hematogones (arrows) resemble<br />

lymphoblasts. They can be distinguished by flow immunophenotyping,<br />

and due to the associated background of small lymphocytes<br />

and regenerating bone marrow.<br />

notyping [22]. Necrosis can be focal or widespread,<br />

and can recur with relapsed disease. Occasional cases<br />

can show bone changes, which include osteoporosis or<br />

osteopenia [23].<br />

In some cases of ALL the principle manifestation of<br />

disease is extramedullary [24]. This is not uncommon<br />

in precursor-T-cell ALL/lymphoma which can present<br />

with a mediastinal mass and lymphadenopathy. Other<br />

sites that may be identified prior to blood and bone<br />

marrow disease include lymph node, skin, testes, and<br />

CNS. Whenever there is concern of a lymphoblastic process<br />

in an extramedullary location, careful review of the<br />

blood and evaluation of the marrow is imperative.<br />

Differential diagnostic considerations based on the<br />

cytomorphologic and histologic features of blasts in<br />

the peripheral blood and marrow depend in part on

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