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Journal of Clinical Oncology, Vol 18, Issue 21 (November), 2000: 3731-3733
© 2000 American Society for Clinical Oncology


DIAGNOSIS IN ONCOLOGY

Two Unusual Lymphomas

Case 1: Primary Malignant Lymphoma (Diffuse Large B-Cell Lymphoma) of the Spleen Mimicking Splenic Abscess

Arthur Skarin MD Consultant Editor

S. Abraksia, P. Dileep Kumar, Jan Kasal

Huron Hospital, Cleveland, OH

A 75-year-old woman presented with fatigue, loss of weight, and intermittent fever and chills of 1 month’s duration. Her examination was significant for a temperature of 38.9°C, conjunctival pallor, absence of lymphadenopathy, and splenomegaly (2 cm below the left costal margin). Significant laboratory data included a hemoglobin level of 7.5 g/dL, hematocrit of 24%, mean corpuscular volume of 67.9 fl, total leukocyte count of 14,800/µL (differential: neutrophils, 88%; lymphocytes, 3%; monocytes, 8%; eosinophils, 1%), and platelet count of 432,000/µL. A peripheral-blood smear showed hypochromia and microcytosis with no atypical or abnormal lymphocytes. An ultrasonogram of the abdomen revealed multiple rounded, predominantly hyperechoic lesions of the spleen. A computed tomographic (CT) scan of the abdomen confirmed these well-circumscribed, rounded, low attenuation lesions (Fig 1). The liver was normal, but retroperitoneal lymphadenopathy up to 2.5 cm was noted. A CT scan of the chest was normal. Splenic abscesses and metastatic tumor were considered in the differential diagnosis. She underwent splenectomy. The spleen weighed 597 g. Gross examination showed multiple irregular lobulated masses, the largest with a diameter of 6.8 cm. Histopathology showed diffuse large-cell lymphoma with uniform sheets of moderately large lymphocytic cells with focally prominent mitotic activity (Figs 2 and 3). There was no involvement of the splenic hilum. Flow cytometric studies were consistent with a B-cell lymphoma (CD2+/CD3+/CD19+/CD20+/CD10-/CD23-/{kappa}-/{lambda}+). A liver biopsy specimen was normal. Bone marrow aspiration and biopsy did not reveal any evidence of lymphoma. Her bone marrow iron level was increased. She died 2 months later after an acute myocardial infarction.



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Fig. 1

 


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Fig. 2

 


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Fig. 3

 
Splenic lymphoma, or primary malignant lymphoma of the spleen (PMLS), is an unusual disease. The true incidence is difficult to estimate because of the variable definitions of the disease. It probably comprises less than 1% of non-Hodgkin’s lymphoma. Some authors consider PMLS to be an entity limited to the involvement of spleen and splenic hilum.1 Others consider PMLS to be an entity that develops in the spleen with the potential for spreading to adjacent organs or distant metastasis.2 The clinical features are characterized by nonspecific systemic symptoms. Most are of B-cell origin, with the most common histologic diagnosis being a low-grade or intermediate-grade lymphoma.3 Splenectomy is still the most effective therapy for all PMLSs.3-5 Although there are no controlled trials, some authors believe that multiagent chemotherapy and/or radiotherapy is useful.6 Cytopenias are common in PMLS, and their reversal early after splenectomy is associated with prolonged survival.7

Single or multiple confluent nodules are seen quite often in diffuse large-cell lymphoma of the spleen.8 Our patient’s clinical presentation, which mimicked splenic abscess, is intriguing. Diffuse large-cell lymphoma of the spleen should be considered in the differential diagnosis of patients presenting with fever or other systemic symptoms associated with radiographic evidence of single or multiple focal lesions in the spleen.

REFERENCES

1. Brox A, Shustik C: Non-Hodgkin’s lymphoma of the spleen. Leuk Lymphoma 11: 165-171, 1993[Medline]

2. Falk S, Stutte HJ: Primary malignant lymphoma of the spleen: A morphological and immunohistochemical analysis of 17 cases. Cancer 66: 2612-2619, 1990[Medline]

3. Gobbi PG, Grignani GE, Pozzetti U, et al: Primary splenic lymphoma: Does it exist? Haematologica 79: 286-293, 1994[Abstract/Free Full Text]

4. Skarin AT, Davey FR, Moloney WC: Lymphosarcoma of the spleen: Results of diagnostic splenectomy in 11 patients. Arch Intern Med 127: 259-265, 1971 [Abstract/Free Full Text]

5. Mulligan SP, Matutes E, Dearden C, et al: Splenic lymphoma with villous lymphocytes: Natural history and response to therapy in 50 cases. Br J Haematol 78: 206-209, 1991[Medline]

6. Narang S, Wolf BC, Neiman RS: Malignant lymphoma presenting with prominent splenomegaly. Cancer 55: 1948-1957, 1985[Medline]

7. Morel P, Dupriez B, Gosselin B, et al: Role of early splenectomy in malignant lymphomas with prominent splenic involvement (primary lymphomas of the spleen). Cancer 71: 207-215, 1993[Medline]

8. Harris NL, Aisenberg AC, Meyer JE, et al: Diffuse large cell (histiocytic) lymphoma of the spleen: Clinical and pathologic characteristics of ten cases. Cancer 54: 2460-2467, 1984[Medline]


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