Unexpected infant death secondary to a pulmonary infiltration due to acute myelocytic leukaemia

Acute myeloid leukaemia (AML) often presents with non-specific symptoms such as fatigue, anaemia or infection. Pulmonary involvement is uncommon in AML during the course of the disease and is usually caused by infection, haemorrhage, leukaemic pulmonary infiltrates and leukostasis. Lung localization of AML is very uncommon and potentially life threatening if not diagnosed and treated rapidly. The authors describe the sudden death of an asymptomatic five-month-infant because of a misdiagnosed lung localization of AML. Autopsy examination followed by histopathological studies showed an extensive leukostasis and extramedullary leukaemic infiltrating the lungs. Special stains and immunohistochemical studies revealed findings consistent with acute myelogenous leukaemia. This case suggests that underlying acute leukaemia should be considered as a cause of flu-like symptoms in infants. Medical personnel are urged to be alert to fever, sore throat, weakness and dyspnea that may be characteristic of serious systemic diseases.

[Cutaneous carcinoma induced by radiotherapy: a report of 31 cases]

Depilatory radiotherapy was used in the sixties as a treatment for ringworm in Tunisia. Subsequently some of these patients developed radio-induced carcinomas of the scalp. To present the epidemiological, clinical, pathological, therapeutic features and out come of radio-induced cutaneous carcinomas. We conducted a retrospective study performed in the dermatology department of the La Rabta hospital of Tunis over a 6- year-period recording all histologically confirmed carcinomas in patients irradiated in childhood for tinea capitis. Thirty one patients were included with 49 tumors: 47 basal cell carcinomas and 2 squamous cell carcinomas. The average latent period between the irradiation and the appearance of the carcinomas was of 35.7 years. The average age was 53 years. A male predominance was noted, with a sex ratio M/F of 6.75. Clinically, basal cell carcinomas were nodular in all cases. Surgery was indicated in 90% of cases. Cryosurgery and radiotherapy were used respectively in 1 and 2 patients. Our study shows that radio-induced cutaneous carcinomas are widely dominated by basal cell carcinoma. They arise, approximately, ten years earlier than carcinoma in patients with no history of scalp irradiation. However X-ray exposure does not seem to influence clinical or histological presentation, therapeutic modalities nor prognosis of these tumors. The prognosis of radioinduced cutaneous carcinomas was globally similar to that of other cutaneous carcinomas with same histological type and equivalent degree of invasion

[Is APRI score a suitable tool for prediction of fibrosis in Tunisian patients with genotype 1 chronic viral hepatitis C?]

Therapeutic indications in chronic genotype 1 hepatitis C are based on severity of fibrosis. APRI score is a simple, cheap and reproducible biochemical test. Performances of APRI score in Tunisian population with chronic hepatitis C were not previously prospectively studied. To evaluate the performances of APRI score in prediction of severity of fibrosis in chronic genotype 1 hepatitis C based on a prospective study. We prospectively include patients with chronic genotype 1 hepatitis C and positive viral load. Hepatic biopsy was performed in all included patients and abnormalities were classified according to METAVIR classification. In all patients, APRI score was calculated based on biochemical data collected within the 15 days before hepatic biopsy. We studied 140 patients [46 men, mean age 48.4 years [20 - 65 years]]. Mean APRI score was 0.89 [0.18 - 3.72]. Statistically significant correlation was observed between APRI score and fibrosis severity [r = 0.31 p < 0.0001]. APRI score was higher in patients with severe fibrosis [F2, F3 or F4] compared to patients with moderate fibrosis [F0 or F1] [0.97 + 0.68 vs 0.62 + 0.44; p 0.009]. Threshold value of APRI score of 0.72 was associated with area under the curve of 0.65 + 0.05 [0.57 - 0.73], sensitivity of 56.3% and specificity of 75.8% in prediction of severe fibrosis. APRI score was also higher in patients with cirrhosis [1.24 + 0.79 vs 0.85 + 0.61; p = 0.01]. Threshold value of 0.86 was associated with area under the curve of 0.69 + 0.07 [0.61 - 0.77], sensitivity of 76.4% and specificity of 65.8% in prediction of cirrhosis. APRI score is not a good alternative to hepatic biopsy although a strong correlation with fibrosis severity, because of relatively low area under the curve, sensitivity and specificity in prediction of severe fibrosis and cirrhosis

[Vasculitis in sweet's syndrome]

Also called acute febrile neutrophilic dermatosis. Sweet's syndrome is non-infective dermatoses that exhibit a predominantly neutrophilic inflammatory infiltrate. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. To describe their clinical, pathological and therapeutic characteristics. We report a series of 47 cases of Sweet's syndrome, collected in our institution between 1997 and 2011. The patient population consisted of 11 males and 36 females. The mean age was 47 years [28-74]. An associated disease process was seen in 10 patients: inflammatory disease [3 cases], inflammatory bowel disease [2 cases], tuberculosis [3 cases] and diabetes [3 cases]. One case of pregnancy was observed. In the prodromal phase, functional symptoms were reported in 38 cases [80.8%]. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominately of neutrophis. Fibrinoid necrosis and intramural inflammation were observed in 8 cases. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's diseases which requires careful surveillance

[Gastric MALT lymphoma: anatomo-clinical data and outcome of early stage H.pylori-associated gastric MALT-lymphoma resistant to treatment]

To describe the clinical, endoscopic and histological particularities of early stage HP associated gastric MALT lymphoma resistant to anti Hp treatment and identify predicting factors of resistance. We retrospectively studied 12 patients with primary low grade gastric localized MALT lymphoma treated with anti HP treatment and diagnosed at La Rabta Hospital from 1999 to 2009. The ultrasonography was normal in 5 patients between the 6 responding patients. Perigastric lymph nodes were found in non responders [33.3%]. Hp eradication was achieved in 66% of patients not responding while Hp was eradicated in 100% of responders. The two non-responding patients with failure of eradication of Hp had a strain resistant to Clarithromycin Hp. Predicting factors of failure of anti HP: HP resistance to antibiotics, the proximal head, and the presence of perigastric lymph nodes. Recently, chromosomal aberrations and immune-histochemical markers have been implicated as factors of non response to anti Hp

[Pseudo tumoral gastric amyloidosis: about a rare case report]

Amyloidosis is an abnormal extracellular deposition of insoluble proteins, which is associated with an involvement of the gastrointestinal tract in 50 to 70% of cases. In primary amyloidosis [light chain amyloidosis], localized gastric involvement is a rare finding which can mimick malignancy. To elucidate the clinical, histological and therapeutic features of pseudo tumoral gastric amyloidosis via a rare report along with a review of related literatures. We report the case of 56-year -old man, admitted with upper digestive outlet obstruction. Linitis plastica with lymph node involvement was suspected by gastroscopy, barium meal and endoscopic ultrasonography but was not confirmed by gastric biopsies. The patient was treated with total gastrectomy with lymph node dissection. Pathological examination demonstrated gastric and lymph nodes amyloidosis and no malignant tumor was found. We propose that amyloidosis should be considered in the differential diagnosis of gastric tumors

[Descriptive epidemiology of extrapulmonary hydatid cysts: a report of 265 Tunisian cases]

Hydatidosis is a parasitic endemic disease in Tunisia. The liver and lung are the most common sites of involvement; however, it can develop anywhere in the body. The aim of the present study was to analyse the epidemiological features of extrapulmonary hydatid cysts and compare our results with those reported in literature. Aretrospective study of 265 extrapulmonary hydatid cysts collected over the 18-year period from 1990 to 2007 was undertaken. There were 101 male and 164 female patients [sex ratio M/F= 0, 61] ranging in age from 2 to 84 years [mean age = 38, 7]. In our series, hydatid cysts involved mainly the kidney [24, 1%], the central nervous system [22, 6%], the liver [19, 6%] and the spleen [11, 3%]. The other less frequent sites included the peritoneum [n = 9], heart [n = 9], bone [n = 6], adrenal gland [n = 4], epiploon [n = 4], orbit [n = 4], ovary [n = 3], prostate [n = 2], bladder [n = 2], breast [n = 2], Douglas' cul-de-sac [n = 2], diaphragm [n = 1], testis [n = 1], broad ligament [n =1], mediastinum [n = 1], nasal cavity [n = 1], soft tissue [n = 1], abdominal wall [n = 1], parotid gland [n = 1], psoas muscle [n =1], synovia [n = 1], thymus [n =1] et le pancreas [n = 1]. In contrast to literature, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which occupies the 3rd rank

[Central nervous system dysgerminoma. A clinicopathological study of 3 cases]

Intracranial germ cell tumors are rarely seen and typically localize in the pineal or suprasellar region. The largest category of germ cell tumors is dysgerminoma. To describe clinicopathological features and immunohistochemical profile of dysgerminomas. We report three cases of central nervous system dysgerminomas. There were two young women and a man who were 6, 11 and 23 year-old. They presented with symptoms of insipidus diabetes [n=3] with association to visual field defects in the third case. Radiological findings showed a supra seller lesion in two cases. Double localization in the pineal and suprasellar regions was seen in the third case. Histologic examination and immunohistochemical study of surgical specimen were consistent with primary central nervous system dysgerminoma