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We report a 41 year old male with sickle cell disease who developed a myelodysplastic syndrome and acute myeloid leukemia with complex karyotype involving chromosomes 5, 7 and 17 after 15 years of hydroxyurea treatment. He responded poorly to induction chemotherapy with cytarabine/idarubicin followed by high dose cytarabine and succumbed to neutropenic sepsis. Multiple systematic reviews, observational studies and clinical trials were conducted to identify the toxicity profile of hydroxurea. Only six cases of leukemia/myelodysplastic syndrome were identified in patients with sickle cell anemia treated with hydroxyurea. Subsequently, it was concluded that hydroxyurea is not leukemogenic. However, it was noted that most of the published studies had only up to 9 years of follow-up. Our patient was started on hydroxyurea in 1990, before the widespread use of the drug and took hydroxyurea for 15 years. His presentation may reflect an outcome otherwise not yet observed because of the short follow-up of prior studies. We believe that the leukemogenic risk of hydroxyurea should be discussed with the patients and their families. Studies evaluating the adverse effects of hydroxyurea should have longer follow-up before definitive conclusions are drawn.

Tumors of salivary glands are uncommon and comprise of about 2%–4% of all head and neck tumors. About 75%–80% of these tumors are benign and include pleomorphic adenoma, monomorphic adenoma, oncocytoma, and papillary cystadenoma lymphomatosum. Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands, representing 5–10% of all salivary gland tumors. Although known to be metastatic to local lymph nodes, distant metastases are rare (especially, with low and intermediate grade tumors). Histologic grade and the expression of various mucin glycoproteins are useful prognostic indicators. We present a case of mucoepidermoid carcinoma of parotid gland origin with distant metastases which is an uncommon occurrence with intermediate grade tumors. Also, this is the first reported case of humoral hypercalcemia of malignancy secondary to mucoepidermoid carcinoma.

IntroductionLaboratory tests play a central role in assessing a patient and orienting the diagnostic evaluation. We report a case where the discrepancy between the manual and automatic cell count gave a hint to the final diagnosis.Case presentationA 55-year-old Caucasian man, known to have hepatitis C, was admitted with acute respiratory failure secondary to acute pulmonary edema and diffuse petechial rash of the lower extremities for the previous 2 months. The initial laboratory tests showed acute renal failure (creatinine of 2.6 mg/dL). During his hospital stay, the patient had a fluctuating white blood cell count with a recorded value of 96,000 cells/mL. On a peripheral smear, the blood cell count was in the normal range. The acute renal failure was secondary to membranoproliferative glomerulonephritis secondary to essential mixed cryoglobulinemia diagnosed by biopsy. The complete blood count values, performed by Beckman/Coulter GenS, were falsely high due to precipitation of plasma cryoglobulins at room temperature. This spurious leukocytosis was previously described in several case reports, but values as high as 96,000 cells/mL were never reported.ConclusionThe presence of cryoglobulins in the blood creates a clinical challenge for the interpretation of several laboratory tests. Pseudoleukocytosis secondary to cryoglobulinemia has been observed in several reported cases with white blood cell counts up to 54,000 cells/mL at room temperature and 85,600 cells/mL at 4°C. If the cryoglobulin precipitates rapidly, aggregated cryoglobulin particles may be interpreted as blood cells. We report the first patient with pseudoleukocytosis secondary to hepatitis C cryoglobulinemia with a spurious leukocytosis of 96,000 cells/mL at room temperature. Other laboratory tests could also be affected: underestimation of true erythrocyte sedimentation rate, pseudothrombocytosis and pseudolymphocytosis. The precipitation can remove the hepatitis C virus and the antibody of cryoglobulins from serum leading to a false negative result. Any discrepancy between the automated and manual white blood cell count should lead to the suspicion of cryoglobulinemia in the clinical setting.

Desmoplastic small round-cell tumor is an uncommon, highly aggressive tumor with a predilection for pediatric age groups and young adults. It is very unusual in the elderly population. Although Agent Orange has been associated with soft-tissue sarcoma, an association with desmoplastic small round-cell tumor has not been reported. A 52-year-old male presented with abdominal distention, dyspnea, and a 9 kg weight loss. Prior history was significant for hepatitis C and diabetes. He was a Vietnam veteran and he admitted being exposed to Agent Orange. On physical examination, the abdomen was distended and tense. Computed tomography scan of the chest, abdomen and pelvis demonstrated extensive mediastinal and retroperitoneal adenopathy, diffuse omental masses and extensive pleural, intra-abdominal and pelvic ascites. Omental core needle biopsy was consistent with desmoplastic small round-cell tumor based on morphology and immunohistochemistry. He responded poorly to chemotherapy with high-dose cyclophosphamide, doxorubicin and vincristine and died 5 months after presentation secondary to neutropenic sepsis despite G-CSF support and antibiotics.