John M. Shaw

Granulocytic sarcoma is associated with the 8;21 translocation in acute myeloid leukemia.

PURPOSE Since the only three cases of granulocytic sarcoma among patients with acute myeloid leukemia (AML) seen at our institution during the last 12 years were each associated with the 8;21 translocation [t(8;21)], we sought to determine if this association is specific and more frequent than previously recognized. PATIENTS AND METHODS We report three patients with AML and t(8;21) who developed granulocytic sarcomas, and review the world literature. RESULTS Between 1980 and 1992, 53 cases of AML French-American-British (FAB) M2 were identified at our institution. Eight (15%) patients had t(8;21). Three of these eight patients (38%) developed granulocytic sarcoma. All three of our patients received conventional intensive antileukemic chemotherapy yet had short relapse-free survival durations. Several series of patients with t(8;21) report that granulocytic sarcomas occur in approximately 18% of this population, which is four times the expected incidence in AML. Thirty-seven cases have been previously reported. Although karyotype analyses were not reported in many cases of granulocytic sarcoma in the literature, the vast majority of abnormal karyotypes in patients with AML involved t(8;21). Recent work with a cell line derived from a patient with t(8;21) indicates that such cells are unusually adherent to culture bottles and are aggregable CONCLUSION Our data suggest that this association is more common than generally recognized and may be specific. Patients with t(8;21) should be observed closely for signs and symptoms of granulocytic sarcoma. These patients may have a less favorable prognosis than other patients with t(8;21). Cooperative oncology groups should retrospectively identify patients with AML and t(8;21) who had a poor outcome to determine if they had a disproportionate incidence of granulocytic sarcoma. If so, aggressive therapy such as bone marrow transplantation may be warranted early in the therapeutic strategy.

Unresectable chemorefractory liver metastases: radioembolization with 90Y microspheres--safety, efficacy, and survival.

PURPOSE To prospectively evaluate the safety, efficacy, and survival of patients with chemorefractory liver metastases who have been treated with yttrium 90 ((90)Y) glass microspheres. MATERIALS AND METHODS Institutional review boards from two institutions approved the HIPAA-compliant study; all patients provided informed consent. One hundred thirty-seven patients underwent 225 administrations of (90)Y microspheres by using intraarterial infusion. Primary sites (origins) included colon, breast, neuroendocrine, pancreas, lung, cholangiocarcinoma, melanoma, renal, esophageal, ovary, adenocarcinoma of unknown primary, lymphoma, gastric, duodenal, bladder, angiosarcoma, squamous cell carcinoma, thyroid, adrenal, and parotid. Patients underwent evaluation of baseline and follow-up liver function and tumor markers and computed tomographic or magnetic resonance imaging. Patients were observed for survival from first treatment. Median survival (in days) and corresponding 95% confidence intervals were computed by using the Kaplan-Meier method. The log-rank statistic was used for statistical significance testing of survival distributions between various subgroups of patients. RESULTS There were 66 men and 71 women. All patients were treated on an outpatient basis. Median age was 61 years. The mean number of treatments was 1.6. The median activity and dose infused were 1.83 GBq and 112.8 Gy, respectively. Clinical toxicities included fatigue (56%), vague abdominal pain (26%), and nausea (23%). At follow-up imaging, according to World Health Organization criteria, there was a 42.8% response rate (2.1% complete response, 40.7% partial response). There was a biologic tumor response (any decrease in tumor size) of 87%. Overall median survival was 300 days. One-year survival was 47.8%, and 2-year survival was 30.9%. Median survival was 457 days for patients with colorectal tumors, 776 days for those with neuroendocrine tumors, and 207 days for those with noncolorectal, nonneuroendocrine tumors. CONCLUSION (90)Y hepatic treatments are well tolerated with acceptable toxicities; tumor response and median survival are promising.

Pharmacokinetic study of a patient with diphenylhydantoin toxicity

Sequential determinations of plasma diphenylhydantoin (DPH) concentration in a patient with DPH toxicity have been used to study its dose‐dependent elimination kinetics. A pharmacokinetic model has been proposed for DPH that provides estimates of the apparent distribution volume, and characterizes the elimination of this drug in terms of a maximal excretory rate and apparent Michaelis‐Menten constant. This three‐parameter model has been used to illustrate a retrospective way of evaluating some possible causes of DPH toxicity in this patient.

Multiple pigmented nail bands during hydroxyurea therapy: An uncommon finding

The pathogenesis of erythema multiforme is presumed to be based on immunologic mechanisms. 6 A dosedependent toxic skin reaction seems unlikely, considering the lack of reports of erythema multiforme during lithium therapy at any serum concentration; our patients concentration was within the accepted therapeutic range. A metabolic idiosyncrasy also seems unlikely in light of the patients previous long-term therapy. We therefore suggest that the reaction was immunologic, ally mediated. Because lithium tablets contain no coloring agents and the excipients used cannot be suspected of being immunogenic, it is probable that lithium itself caused the cutaneous reaction.

Erythema gyratum repens unassociated with internal malignancy

A case report of erythema gyratum repens occurring in a 68-year-old man is presented. Evaluation and follow-up for development of malignancy over a 39-month period failed to reveal evidence of malignancy. The patient died of an unrelated cause. Autopsy did not demonstrate any evidence of malignancy.

Multicentric reticulohistiocytosis with salivary gland involvement and pericardial effusion

A 60-year-old Palestinian woman developed extensive cutaneous lesions of multicentric reticulohistiocytosis. She also developed a pericardial effusion and involvement of the submandibular salivary glands. Soft tissue gallium images demonstrated this systemic involvement, and this procedure is proposed as a screening method to assess the extent of the disease. The patient showed partial remission after being treated with prednisone, vincristine sulfate, and cyclophosphamide.

Hairy Cell Leukemia With Marked Lymphocytosis

Hairy cell leukemia (HCL) is a rare small B-cell lymphoproliferative disorder. The neoplastic cells have round to oval nuclei and abundant cytoplasm with ‘‘hairy’’ projections seen in the peripheral blood and bone marrow. They typically diffusely infiltrate bone marrow and spleen. Immunophenotypically, they strongly express CD103, CD22, CD11c, and CD25. Hairy cell leukemia commonly presents with pancytopenia and splenomegaly with few circulating neoplastic cells. We describe the case of a 42-year-old man who presented at our institution with marked leukocytosis (white blood cell count, 98 300/mL) and splenomegaly. The patient had no significant past medical history. His hemoglobin level was 9.9 g/dL and his platelet count was 154 000/mL. Review of a peripheral blood smear demonstrated marked lymphocytosis consisting of larger than usual lymphocytes with round to oval, eccentrically located nuclei; small inconspicuous nucleoli; and relatively abundant cytoplasm (Figure 1). Most of the lymphocytes displayed cytoplasmic projections (Figure 1, inset). Many of the cells were tartrate-resistant acid phosphatase (TRAP) positive (Figure 2). A normochromic, normocytic anemia with occasional ovalocytes and teardrop cells was also noted. The bone marrow core biopsy was markedly hypercellular (90%), with most of the medullary space occupied by small to medium-sized, monotonous-appearing lymphocytes with abundant cytoplasm (Figure 3). Flow cytometric analysis performed on the bone marrow aspirate demonstrated these cells to be k-light chain restricted and CD191, CD201, CD52, CD102, CD11c1, CD1031 ,a nd CD25 1 . The differential diagnosis of the peripheral blood smear in this case would include HCL; chronic lymphocytic leukemia; prolymphocytic leukemia; the leukemic phase of mantle cell, follicular, and marginal zone lymphomas; and the rare Japanese variant of HCL (HCLV). The morphology, cytochemistry, and immunophenotype (CD19 1 , CD20 1 , CD11c 1 , and CD103 1 ) of the neoplastic cells in our case are diagnostic for classic HCL, although the high lymphocyte count is a very unusual finding. The HCL variant, although extremely uncommon, does typically present with a marked lymphocytosis (usually around 50 000/mL). However, the neoplastic cells in HCLV typically have prominent nucleoli (resembling prolympho