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19 Mature lymphoproliferative disorders

Mature lymphoproliferative disorders frequently are derived from a single clone of cells. Although this group of diseases involves lymphocytes, the morphological presentation is variable. The integration of clinical and morphological disease features with immunophenotyping is necessary for appropriate recognition and classification. Only representative samples are included in this atlas.

NOTE: Sustained absolute lymphocytosis in an adult should be investigated to differentiate reactive from malignant processes. Characteristics of reactive lymphocytes are listed in Table 14-1.

Chronic lymphocytic leukemia

FIGURE 19–1A Small lymphocytes with smudge cells at arrows (PB ×500).

FIGURE 19–1B Albumin smear-same patient as presented in figure 19-1A (PB ×500).

FIGURE 19–1C Small lymphocytes with smudge cell (PB ×1000).

FIGURE 19–1D (BM ×500).

Morphology

Peripheral blood:

Mature-appearing lymphocytes with round nuclei and block-type chromatin; inconspicuous nucleoli, scant cytoplasm; homogeneous appearance within a given patient; lymphocytes more fragile than normal, leading to “smudge” cells

Absolute sustained lymphocytosis

± Normocytic normochromic anemia (approximately 10% of patients develop an autoimmune hemolytic anemia)

± Thrombocytopenia

Bone marrow:

≥30% lymphocytes

Immunophenotype

CD20⁺, CD19⁺, CD5⁺, CD23⁺

Note:

Addition of albumin to blood prior to smear preparation stabilizes CLL cells, decreasing the formation of smudge cells and allows for accurate cell classification.

B cell prolymphocytic leukemia

Morphology

FIGURE 19–2A (PB ×500).

FIGURE 19–2B (PB ×1000).

FIGURE 19–2C (BM ×500).

FIGURE 19–2D (BM ×1000).

Peripheral blood:

Medium-sized cells (approximately twice the size of small lymphocyte), one prominent nucleolus, moderately condensed chromatin; small-to-moderate slightly basophilic cytoplasm

Absolute lymphocytosis, usually >100 × 10⁹/L

Anemia

Thrombocytopenia

Bone marrow:

Predominantly prolymphocytes with few residual hematopoietic cells

Immunophenotype

CD20⁺, CD19⁺, FMC7⁺

Hairy cell leukemia

FIGURE 19–3A (PB ×500).

FIGURE 19–3B (PB ×1000).

FIGURE 19–3C (BM ×500).

FIGURE 19–3D (BM ×1000).

Morphology

Peripheral blood:

Small- to medium-sized lymphocytes, reniform to oval nucleus with diffuse homogeneous chromatin, ± nucleolus, cytoplasm irregular, and gray-blue hairlike projections

Pancytopenia

Bone marrow:

Aspirate difficult to obtain because of marrow fibrosis (dry tap), cells more easily distinguished by phase or electron microscopy

Immunophenotype

CD19⁺, CD20⁺, CD22⁺, CD11c⁺, Annexin A1⁺

Plasma cell myeloma

FIGURE 19–4A Plasma cells. Note rouleaux (PB ×500).

FIGURE 19–4B Plasma cells (PB ×1000).

FIGURE 19–4C Plasma cells, one multi-nucleated (BM ×500).

FIGURE 19–4D Plasmablast (BM ×1000). Note the lighter blue cytoplasm with the indistinct hof and the slightly eccentric nucleus with 2 distinct nucleoli.

FIGURE 19–4E Proplasmacyte (PB ×1000). The cytoplasm is darker blue and the perinuclear hof is distinct. The nucleus is eccentric and the nucleolus is partially masked by the clumped chromatin.

FIGURE 19–4F Flame cell (BM ×1000). Associated with plasma cells that are producing IgA.

FIGURE 19–4G Mott cell (Morula cell, grape cell) (BM ×1000). Plasma cell containing multiple round globules of immunoglobulin, which stain pink, colorless, or blue.

Morphology

Peripheral blood: rouleaux, rare circulating plasma cell; ± neutropenia

Note:

>2 × 10⁹/L circulating plasma cells suggest plasma cell leukemia.

± Normocytic, normochromic anemia

± Thrombocytopenia

Note:

The background of Wright-stained blood smears may be blue because of abnormal amounts of immunoglobulin.

Bone marrow:

>10% plasma cells, often >30%

Immature ± larger than normal plasma cell with increased N/C ratio; abnormal nuclear chromatin; ± nucleoli, ± multinucleated

Cytoplasm pale blue or dark; cytoplasm may contain immunoglobulin inclusions

Immunophenotype

CD19⁻, CD38⁺, CD138⁺

Note:

This disease may be distinguished from Waldenström macroglobulinemia and heavy chain disease by immunoelectrophoresis.

Burkitt leukemia/lymphoma

FIGURE 19–5A (PB ×500).

FIGURE 19–5B (PB ×1000).

FIGURE 19–5C (BM ×500).

FIGURE 19–5D (BM ×1000).

Morphology

Peripheral blood:

Medium- to large-sized cells with dark blue vacuolated cytoplasm, inconspicuous nucleoli

Bone marrow:

Monotonous pattern of deeply basophilic cells with vacuolated cytoplasm

Immunophenotype

CD5⁻, CD20⁺, CD19⁺, CD10⁺

Lymphoma

FIGURE 19–6A Cleaved lymphoma cells (PB ×1000).

FIGURE 19–6B Large lymphoma cell (PB ×1000).

FIGURE 19–6C Lymphoma cell with prominent nucleoli (PB ×1000).

FIGURE 19–6D “Flower” nucleus suggestive of T-cell lymphoma (PB ×1000).

Morphology

Peripheral blood:

Representative examples of lymphoma cells occasionally observed in peripheral blood.

Bone marrow:

NA

Note:

The diagnosis of lymphoma is determined by lymph node biopsy, immunophenotyping, and molecular genetics.

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19 Mature lymphoproliferative disorders

Mature lymphoproliferative disorders frequently are derived from a single clone of cells. Although this group of diseases involves lymphocytes, the morphological presentation is variable. The integration of clinical and morphological disease features with immunophenotyping is necessary for appropriate recognition and classification. Only representative samples are included in this atlas.

NOTE: Sustained absolute lymphocytosis in an adult should be investigated to differentiate reactive from malignant processes. Characteristics of reactive lymphocytes are listed in Table 14-1.

Chronic lymphocytic leukemia

FIGURE 19–1A Small lymphocytes with smudge cells at arrows (PB ×500).

FIGURE 19–1B Albumin smear-same patient as presented in figure 19-1A (PB ×500).

FIGURE 19–1C Small lymphocytes with smudge cell (PB ×1000).

FIGURE 19–1D (BM ×500).

Morphology

Peripheral blood:

Mature-appearing lymphocytes with round nuclei and block-type chromatin; inconspicuous nucleoli, scant cytoplasm; homogeneous appearance within a given patient; lymphocytes more fragile than normal, leading to “smudge” cells

Absolute sustained lymphocytosis

± Normocytic normochromic anemia (approximately 10% of patients develop an autoimmune hemolytic anemia)

± Thrombocytopenia

Bone marrow:

≥30% lymphocytes

Immunophenotype

CD20⁺, CD19⁺, CD5⁺, CD23⁺

Note:

Addition of albumin to blood prior to smear preparation stabilizes CLL cells, decreasing the formation of smudge cells and allows for accurate cell classification.

B cell prolymphocytic leukemia

Morphology

FIGURE 19–2A (PB ×500).

FIGURE 19–2B (PB ×1000).

FIGURE 19–2C (BM ×500).

FIGURE 19–2D (BM ×1000).

Peripheral blood:

Medium-sized cells (approximately twice the size of small lymphocyte), one prominent nucleolus, moderately condensed chromatin; small-to-moderate slightly basophilic cytoplasm

Absolute lymphocytosis, usually >100 × 10⁹/L

Anemia

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Clinical Hematology Atlas