ABSTRACT.We describe the complex presentation of a patient with renal medullary carcinoma, a newly described entity primarily affecting young patients with sickle cell trait. Renal medullary carcinoma is an aggressive, rapidly destructive tumor associated with a delayed diagnosis and a poor outcome. The most common presenting signs and symptoms include hematuria, abdominal or flank pain, and weight loss. Sickle cell trait as the sole cause of hematuria in young black patients is a diagnosis of exclusion. Hemoglobin electrophoresis, intravenous pyelography, and computed tomography scans should be the minimal studies performed in young black patients with hematuria. Pediatrics 1999;103(2). URL: http://www.pediatrics.org/cgi/content/full/103/2/e22; sickle cell trait, hematuria, renal medullary carcinoma, renal tumors.ABBREVIATION. CT, computed tomography. R enal tumors represent 7.8% of all pediatric malignancies in the United States.1 Most of these are Wilms' tumors affecting children Ͻ5 years of age. Recently, a new entity, renal medullary carcinoma, has been described in young people, including children, with sickle cell disorders.2 Many of the reported cases have been diagnostic challenges with poor prognoses. It is unclear whether earlier diagnosis and earlier treatment will result in a better prognosis, but awareness of this newly described entity is most important. We describe an 18-year-old female with sickle cell trait who was diagnosed with renal medullary carcinoma after a complex presentation. CASE PRESENTATIONW.B. was an 18-year-old black female with known sickle trait, who was in good health until 6 months before admission when she developed swelling and stiffness in her metacarpophalangeal and knee joints. Her initial evaluation was significant for microscopic hematuria and a positive antinuclear antibody test with a titer of 1:640 and a diffuse pattern. She was treated with oral salicylates and naproxen sodium in the 2 months before this admission, but she took them infrequently.Two months after her initial presentation, she developed daily nausea and bilious vomiting, resulting in a 20-lb weight loss (15% of total body weight) over a 3-week period. She sought medical care when she developed worsening right-sided flank pain and gross hematuria. She was admitted with a presumptive diagnosis of papillary necrosis. Her review of systems at the time of admission was also significant for fever, sharp intermittent periumbilical pain, midline back pain, right scapular pain, urinary frequency with nocturia, gross hematuria with clots, loose stools, and night sweats. She denied dysuria. The patient had a past medical history of hypertension of unknown cause (untreated), epistaxis, easy bruising, and vaginal yeast infections. She had been sexually active for 4 years.At the time of physical examination, the patient was well developed and appeared well nourished despite her weight loss. She was bent over, secondary to pain. Her vital signs were significant for a blood pressure of 158/87 mm Hg. She had two café ...
A four-year-old child with recurrent infections and increasing hepatosplenomegaly over a three-year period was evaluated. Increased numbers of myeloid precursors packed the bone marrow and infiltrated the peripheral blood. A diagnosis of chronic myelogenous leukemia (CML) was considered but could not be confirmed by laboratory studies appropriate for the types of CML usually observed in childhood. Examination of the patient's peripheral blood smears revealed many atypical monocytoid cells with unipolar hairy projections. Scanning electron microscopy showed these to be leukemic monoblasts with characteristic broad-based ruffles on the cell surface. A population of myeloid precursors possessing narrow ridge-like profiles was also observed. Progressive infiltration of the spleen caused hypersplenism which necessitated splenectomy. Subsequently, massive liver and bone marrow involvement led to the patient's death. Terminally, the proliferating blast cells were demonstrated to be leukemic monoblasts by analysis of cytochemical staining patterns, surface immunoglobulins, serum lysozyme levels, and monocyte-mediated antibody-dependent cellular cytotoxicity studies. The findings in this case are most compatible with a diagnosis of chronic myelomonocytic leukemia (CMML), a condition not previously described in childhood. Several myeloproliferative disorders with prolonged survival have been reported in children, but special studies were not performed to determine which cell lines were abnormally proliferating. The similarities between these children and our patient with CMML suggest that monocyte studies may be useful in the diagnosis of these unusual disorders, provide insights into their pathogenesis, and aid in the selection of appropriate therapy.
Transient myeloproliferative disorder (TMD) and subsequent acute myeloid leukemia (AML) occur with increased frequency in infants and children with Down syndrome. TMD can also occur in phenotypically normal newborns. We describe the second case of a non-Down syndrome child with TMD who subsequently developed AML. Trisomy 21 karyotypic was restricted to hematopoietic cells in the blood and bone marrow. No other karyotypic abnormalities were found. Leukemic blasts showed megakaryoblastic features with immunophenotyping. This case shows that TMD in a child without Down syndrome may not be entirely benign. Close follow-up is warranted.
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