Abdullah Avni Atay

Sinus histiocytosis with massive lymphadenopathy in three brothers

Rosai-Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy; SHML), is a rare cause of lymph node enlargement in children. 1 It consists of chronic massive enlargement of cervical lymph nodes frequently accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR), and hyperglobulinemia. 2,3 Extranodal sites are involved in approximately 25% of patients. 3 Although elevated antibody titers to some viruses have been found, evidence to incriminate these as etiologic agents is lacking. 4–6 To date, neither a definite etiologic agent nor a genetic inheritance has been identified in cases of SHML. It is known as a nonfamilial disease of the lymphoreticuloendothelial system. This report describes three brothers with SHML and this is the first report of three cases in one family.

Multifocal peliosis hepatis: MR and diffusion-weighted MR-imaging findings of an atypical case

Abstract Peliosis is a rare benign disorder that is characterized by the presence of diffuse blood-filled cystic spaces and can occur in the liver, spleen, bone-marrow, and lungs. We present a 10-year-old boy with Fanconi anemia who presented with peliosis hepatis due to androgen treatment. Magnetic resonance (MR) imaging revealed multiple non-enhancing masses. Some of the lesions revealed fluid-fluid levels and extrahepatic extension on MR images. Diffusion-weighted (DW) imaging showed restricted diffusion. Fluid-fluid levels and extrahepatic extensions are unusual findings for hepatic peliotic lesions. In addition, DW imaging findings of peliosis hepatis have not been reported previously.

Ceftriaxone-related hemolysis and acute renal failure

A 5-year-old girl with no underlying immune deficiency or hematologic disease was treated with a combination of ceftriaxone and ampicilline-sulbactam for pneumonia. On the ninth day of the therapy, she developed oliguria, paleness, malaise, immune hemolytic anemia (IHA) and acute renal failure (ARF). Laboratory studies showed the presence of antibodies against ceftriaxone. Acute interstitial nephritis (AIN) was diagnosed by renal biopsy. The patient’s renal insufficiency was successfully treated with peritoneal dialysis without any complications. The patient recovered without any treatment using steroids or other immunosuppressive agents.

Mycoplasma pneumoniae infection associated with pancytopenia: a case report.

Immune hemolytic anemia is a rare condition in childhood. Cold agglutinins have been implicated in the etiology of the hemolysis and frequently observed during Mycoplasma pneumoniae infections. We present here a case of cold agglutinin-related hemolytic anemia, thrombocytopenia, and leukopenia secondary to M. pneumoniae associated pneumonia. It is suggested that even though very rare, M. pneumoniae infection should be considered as the underlying disease in a patient presenting with pancytopenia.

Stroke associated with pseudoephedrine in a child with rhabdomyosarcoma

Pseudoephedrine is a nasal vasoconstrictor and is contained in many cough and cold medications. It is generally harmless when used in recommended doses. Hypertensive crisis, psychosis, hemorrhagic stroke, and intracranial hemorrhage have been reported as severe complications. We report a 4-year-old girl with nasopharyngeal rhabdomyosarcoma who developed stroke while on pseudoephedrine therapy for 3 days and had no hematological abnormalities.

Panniculitis induced by a chemotherapy regimen consisting of topotecan and cyclophosphamide.

To the Editor: Drug-induced panniculitis is uncommon and has been reported secondary to methotrexate, potassium bromide, apomorphine, and recombinant agranulocyte-colony-stimulating factor [1–4]. Panniculitis induced by mitoxantrone, ifosfamide, vinorelbine, and etoposide (MINE) chemotherapy had been reported in patients with Hodgkin Disease [5,6]. To date, there has been little information available about association of panniculitis with chemotherapy regimens including topotecan and cyclophosphamide, in pediatric patients. An 11-month-old boy diagnosed with abdominal germ cell tumor had been treated with a chemotherapy regimen consisting bleomycin, etoposide, and cisplatin. After seven courses, a good response was achieved. Therapy was changed to ICE (ifosfamide, etoposide, and carboplatin) after relapse, radiotherapy was also administered. There was no response after two cycles. Topotecan was administered at 2 mg/m/day (2nd and 3rd days) by continuous infusion over 24 hr and cyclophosphamide administered intravenously at 2,100 mg/m/ day (2nd, 3rd, and 4th days) with Uromitexan (MESNA) repeated every 3 weeks. Six day after the second course of this schedule, the abdominal mass shrunk 25% but three skin nodules appeared which were below the left lower eyelid, under the right nipple, and at the intersection of midclavicular line and the ninth rib. These lesions were approximately 2 2 cm in diameter, dull red, edematous, somewhat tender, and fixed to the overlying skin. Histopathological examination of the nodules revealed septal panniculitis. These violaceous nodules disappeared in 2 weeks. Serum lipase, antitrypsin, and amylase were normal as was urine vanilyl mandelic acid. Antinuclear antibody, anti-dsDNA, and anti-Sm antibody were negative. Two months after the appearance of the skin lesions the patient died of the progressive disease. Despite the progression of the disease, the nodules did not reappear. Most paraneoplastic dermatoses disappear when the primary tumor is removed and reappear in the case of recurrence or metastases of the cancer. The clinical course of panniculitis related to malignancy is also parallel to that of the primary tumor, and treatment of the underlying malignancy often results in regression of the panniculitis. Panniculitis was not secondary to the disease in our case. Hasegawa et al. had reported a patient with intracranial germ cell tumor associated with panniculitis [4]. However, they showed that the dermatologic lesions were secondary to the recombinant granulocyte-colony-stimulating factor treatment. Potassium bromide, apomorphine, interferonalpha, interleukin-2, methotrexate, and corticosteroids had also been reported to be the responsible agents from panniculitis. Panniculitis was induced by a regimen including of topotecan, and cyclophosphamide in our patient. After administration of this combined therapy, the patient developed panniculitis which subsequently resolved spontaneously. Saint-Cyr et al. reported panniculitis induced by a chemotherapy regimen consisting MINE [5]. Maubec et al. also reported subcutaneous inflammatory edema induced by MINE chemotherapy [6]. Both reports used MESNA to protect the patient from adverse effects of cyclophosphamide. We have also used MESNA in our patient which could also be the responsible agent for panniculitis. Although the etiologic agent is unclear in our patient, it is important that topotecan, cyclophosphamide, and MESNA be kept in mind in malignancies in which panniculitis develops.

Nephrogenic diabetes insipidus due to a novel AVPR2 mutation.

Congenital nephrogenic diabetes insipidus (CNDI) is a rare inherited disease characterized by renal tubular unresponsiveness to the antidiuretic effect of arginine-vasopressin due to the mutations of two molecules, the vasopressin V2 receptor (AVPR2) and the aquasporin-2 water channel. We report a novel AVPR2 mutation in a Turkish 18 month-old boy with skeletal anomalies.

Effects of Recombinant Granulocyte Colony-Stimulating Factor and Granulocyte-Macrophage Colony-Stimulating Factor on Platelet Aggregation in Healthy Volunteers

ABS TRACT Objective: Recombinant human granulocyte colony-stimulating factor (G-CSF) is increasingly used for stem cell mobilization in healthy donors for allogeneic stem cell transplantation. However, a possible association between thrombosis and G-CSF administration has been reported. This study was performed to investigate the in vitro effects of G-CSF and granulocyte-macrophagecolony-stimulating factor (GM-CSF) on platelet aggregation in healthy volunteers. Material and Methods: Platelet aggregation was induced by adenosinediphosphate (ADP) and collagen in platelet rich plasma (PRP) and whole blood (WB) samples from 26 healthy volunteers (20 volunteers for G-CSF, and 6 volunteers for GM-CSF study). Three concentrations of G-CSF solution (10, 50 and 100 ng/mL) and GM-CSF (5, 10 and 20 ng/mL) were prepared. Each concentration of G-CSF and GM-CSF solutions and the control diluent were incubated with PRP and WB samples. After incubation, aggregation responses were evaluated with ADP (1 μM and 5 μM) and collagen (1 μg/mL) in PRP and WB samples. Results: We found that G-CSF increased ADP and collagen induced platelet aggregation in PRP and ADP induced platelet aggregation rate in WB. GM-CSF didn’t affect ADP and collagen induced platelet aggregation rate in whole blood and platelet-rich plasma in vitro. Conclusion: This study showed that G-CSF administration may lead to platelet hyperaggregability. The enhancing effect of G-CSF on platelet aggregation should be taken into consideration in clinical usage.