Q fever as a rare cause of hemophagocytic lymphohistiocytosis: Case report

Hemophagocytic Lymphohistiocytosis (HLH) is a reactive disorder of the mononuclear phagocytic system characterized by increased histiocytic proliferation, activation and hemaphagocytosis. The underlying etiology may be genetic (primary) or acquired (secondary). Secondary causes include drugs, autoimmune diseases, malignancies and infections of which EBV is the most common. A 28-year old male patient who was a shepherd with no known concomitant comorbid disease was admitted to the Emergency Department with the complaints of abdominal pain, fever, severe fatigue. Physical examination revealed high fever, hepatosplenomegaly and laboratory examination revealed pancytopenia, hyperferritinemia and hypertriglyceridemia. Hemophagocytes were observed in the bone marrow biopsy and the patient was diagnosed as HLH. The patient was treated with cyclosporine A, dexamethasone, intravenous immunoglobulin (IvIg) and etoposide according to the HLH 2004 protocol. Coxiella burnetii was detected in the serological evaluation of the etiology and doxycycline was added to the current treatment. Fever was controlled in the second week of the treatment and the patient was discharged after complete recovery of the cytopenia in the fourth week. In the outpatient setting, treatment was completed in 8 weeks and follow-up of the patient is still ongoing without medication. To the best of our knowledge, this is the first case from Turkey of HLH secondary to Q-fever which was treated and managed successfully. Since the mortality of HLH is quite high, the etiology should be determined as soon as possible to be able to provide appropriate treatment.     

木村病1例报告并文献复习

目的探讨木村病的临床特点、临床诊断以及临床治疗。方法分析房山区第一医院2009-02诊断治疗的木村病患者1例的临床资料。结果木村病青、中年男性多见,一般病程较长,临床主要表现为颌下、腮腺及耳后区的慢性无痛性肿块,同时伴外周血嗜酸性粒细胞明显增高,占白细胞总数的28%~65%。组织病理学以淋巴细胞浸润、血管增生及局部嗜酸性粒细胞浸润为特点,手术、放射和类固醇激素治疗有效,预后较好。结论木村病为罕见病,加强对其诊断的认识,可以得到有效的治疗。     

Acute Q fever in southern Taiwan: atypical manifestations of hyperbilirubinemia and prolonged fever

The clinical information of acute Q fever in Taiwan was limited. A clinical study of 109 adults with serologically documented acute Q fever in the past decade (1994-2005) at 3 referral hospitals in southern Taiwan was reported. Their clinical manifestations, laboratory findings, and clinical outcomes were analyzed. Males predominated (98, 90%). There is a significant correlation between monthly average temperature and case numbers of acute Q fever (r = 0.74, P = 0.006). Fever (99%), chills (69%), and headache (45%) were the common symptoms, and relative bradycardia (44/60, 73 %) was often noted. Acute hepatitis, defined as either serum aspartate aminotransferase >or=60 IU/L or alanine aminotransferase >or=78 IU/L, was found in 88 (85%) cases, and more than one-third (31/87, 36%) had hyperbilirubinemia (serum total bilirubin >or=1.4 mg/dL) at initial presentation. The intervals between initiation of appropriate therapy to defervescence were longer in patients with hyperbilirubinemia than those without hyperbilirubinemia, irrespective of tetracycline or fluoroquinolone therapy. Of note, 8 (7.3%) cases experienced a prolonged period of fever (>28 days). In southern Taiwan, the predominant presentation of acute Q fever is acute febrile illness with hepatitis with or without jaundice. Acute Q fever should be added to the list of differential diagnoses of patients with fever, headache, relative bradycardia, elevated serum aminotransferase levels, or prolongation of activated partial thromboplastin time, irrespective of jaundice.     

Transient visual impairment in a patient with psychogenic fever: A case report

BackgroundPsychogenic fever is a stress-related psychosomatic disease and has been recognized clinically since the early twentieth century. It is common in adolescents or young women with acute or persistent elevation in body temperature. Psychogenic fever can be attenuated by anxiolytic, neuroleptic, or by psychotherapy.Case summaryA 22-year-old female was diagnosed with psychogenic fever and suffered from fever, headache and transient visual impairment. She had a history of being hit on the head by a rolling gate 10 days prior to visiting our hospital. All examinations showed no abnormal findings. Antipyretic drugs and antibiotics had no effect on reducing her body temperature. The patient was usually nervous at work and became extremely anxious after the injury. Within a week of psychotherapy, her temperature gradually returned to normal and her sight was restored.ConclusionPsychological stress may present with a range of symptoms, including visual impairment. Physicians should consider the diagnosis of psychogenic fever when patients present with fever of unknown origin and no other abnormal findings.     

Spinal cord toxoplasmosis as an unusual presentation of AIDS: case report and review of the literature

Approximately 10% of patients with AIDS present with some neurological deficit as their initial complaint, and up to 80% will have CNS involvement during the course of their disease. Toxoplasmosis is the most common cause of cerebral mass lesions in patients with AIDS, but appears to be an uncommon cause of spinal cord disease. The incidence of myelopathy may be as high as 20%, with 50% of the cases reported post-mortem. We present a unique case of spinal cord disease as the initial presentation of AIDS. We also present a comprehensive literature review of this topic, its diagnosis and treatment. This is a retrospective chart review case report. After a detailed case presentation, several diagnostic and therapeutic aspects of this unique case are thoroughly discussed. Although spinal cord toxoplasmosis is uncommon, it has been suggested that most patients with AIDS that present with evolving myelopathy, characterized by extremity weakness, sensory involvement, spinal cord enlargement, enhancing lesions in brain or spinal cord CT or MRI, have toxoplasmic myelitis.     

Metal fume fever: a case report and review of the literature

Metal fume fever is an acutely noxious inhalation syndrome secondary to metal oxide fumes. Despite preventative strategies sporadic cases are likely to continue to present to emergency departments.     

Diagnosis and treatment of Q fever: attempts to clarify current problems in Japan

“Q fever” is a generic term for infection caused, mostly in the form of pneumonia or bronchitis, by Coxiella burnetii (Q-fever Coxiella), a pathogen closely related to Rickettsia and Legionella. Q fever is an influenza-like, transient febrile infectious disease that is common to humans and animals; it develops after the transmission of the infectious agent from livestock or pet animals, but person-toperson transmission is rare. In Europe and the United States, it is ranked fourth or fifth as an underlying cause of community-acquired pneumonia. Many patients with Q fever have a good prognosis, and their mortality is about 1%–2% when left untreated. However, because some patients may take a long time to be cured or may have a chronic condition with poor prognosis, patients with definitely diagnosed Q fever or those strongly suspected of having Q fever are strongly recommended to receive treatment. The definite diagnosis of Q fever is made based on a significant increase in serum antibody titers, the determination of which often requires considerable time, and therefore patients must be monitored for a certain period. Q-fever Coxiella, an obligate intracellular parasite, is basically not susceptible to β-lactam antibiotics, which barely permeate into the cells, but the parasite is susceptible to tetracyclines, macrolides, and quinolones, with these agents being sufficiently permeable into the cells. However, there are many cases of spontaneous cure, and it is likely that β-lactam treatment may have been involved in these cases. Vaccination against Q fever is not common in Japan.     

Rh iso-immunisation with syndrome of hepatocellular damage. An unusual presentation with review of literature

Rh iso-immunisation is prevalent in many underdeveloped countries. Severe haemolytic anaemia with or without hydrops fetalis, dangerous level of haemolytic unconjugated bilirubin and imminent bilirubin encephalopathy are the hallmarks of haemolytic disease of the newborn. The investigative protocols and efficient management of this entity are adequately described in literature. An unusual manifestation of this disease with severe hepatocellular damage and conjugated hyperbilirubinemia was noticed in a neonate. The literature has been reviewed and the specific symptom complex has been attributed to 'syndrome of hepatocellular damage', which is a rare accompaniment of haemolytic disease of the newborn. Attempts have been made to describe the syndrome in detail, with latest reference regarding complete workup and management. Few useful tips for prevention of the disease in a community background have been suggested.     

Autoimmune polyglandular syndrome type 3: A case report of an unusual presentation and literature review

Autoimmune polyglandular syndromes (APS) are rare disorders characterized by auto‐destruction of endocrine and non‐endocrine organs by organ‐specific antibody‐directed T‐lymphocytic infiltration. This case highlights a 29‐year‐old Caucasian man with vitiligo found to have significant neurological abnormalities in the setting of newly diagnosed pernicious anemia and thyroid autoimmune disease.     

Multiple mycotic hepatic and splenic artery aneurysms in a patient with pneumococcal pneumonia: a case report with a review of the literature

It is well‐known that the major organisms for mycotic aneurysms are Staphylococcus aureus, Salmonella spp but is extremely rare in Streptococcus pneumoniae in postantibiotic era. We demonstrated the first case with multiple mycotic aneurysms simultaneously generated in the splenic and hepatic arteries in a patient with pneumococcal pneumonia.     

Basilar artery thrombosis in a trauma patient. Case report and review of the literature

Thrombotic disease of the vertebrobasilar circulation is associated with a poor prognosis. It may occur in trauma patients, especially those with neck injuries and even several months after the initial insult. We report on the case of a young polytrauma patient, victim of a traffic accident, with associated cervical and thoracic spinal injuries resulting in paraplegia. Consciousness was not impaired initially, but during transfer to our hospital he became suddenly unconscious. An occluded basilar artery was found on angiography, but unfortunately we were unable to reopen the vessel with thrombolytic therapy. This case again proves that lesions of the vertebro-basilar system must always be suspected in neck injuries. Even after minor whiplash injuries, fatal basilar thrombosis may occur. A review of all reported cases of traumatic basilar artery thrombosis is given and the use of thrombolytic therapy is discussed.     

Large infratentorial cystic oligodendroglioma in a pregnant patient: A case report of a rare presentation and literature review

Oligodendrogliomas (ODGs) are rare brain tumors in adults, mostly presenting as a supratentorial solid mass, while less than 10% occur infratentorially. Infratentorial cystic ODGs are extremely rare. We reported a large partially cystic cerebellar ODG in a 31‐week pregnant patient with an unusual presentation and discussed the challenging management.     

Case report and literature review: transient Inab phenotype and an agglutinating anti-IFC in a patient with a gastrointestinal problem

BACKGROUND: The Inab phenotype is a rare deficiency of all Cromer antigens. These antigens are carried on the decay-accelerating factor (DAF, CD55) molecule that is attached to the red blood cell (RBC) membrane by a glycosylphosphatidylinositol (GPI) anchor. Although typically inherited, an acquired and transient form of the Inab phenotype also exists. A patient with the triad of transient Inab phenotype, a direct-agglutinating anti-IFC, and gastrointestinal (GI) abnormalities is reported. CASE REPORT: An 18-month-old boy with gastroesophageal reflux disease requiring a feeding tube, milk and soy intolerance, and severe growth retardation, as well as vision and hearing deficits from cytomegalovirus infection, was identified when pretransfusion testing revealed a potent panagglutinin (titer > 2000 at 4 degrees C). This antibody did not react with Dr(a-) and IFC RBCs, and the autocontrol was negative. The patient's RBCs lacked CD55 by flow cytometric techniques but had normal levels of CD59 and antigens such as Yt(a) and Emm, carried on GPI-linked proteins, thus excluding paroxysmal nocturnal hemoglobinuria. Several months after initial detection, the anti-IFC was virtually undetectable and his cells reacted weakly with anti-IFC, anti-Dr(a), and anti-CD55. RBCs from the propositus' parents and brother demonstrated normal CD55 and CD59 expression. CONCLUSION: This is the first example of a direct-agglutinating anti-IFC. The cause of the transient depression in CD55 protein (and thus Cromer system antigens) and appearance of anti-IFC remains unknown, as does the relationship between the patient's GI system abnormalities and these serologic findings.     

Bacterial peritonitis in a patient with malignant ascites caused by pancreatic carcinoma: Case report and review of literature

Bacterial peritonitis, an infection of the ascitic fluid, can be classified etiologically as spontaneous or secondary bacterial peritonitis. The former is mainly caused by portal hypertension and its subsequent effects, whereas the latter is caused by the direct dissemination of bacteria into the peritoneal cavity. Previous reports have described some distinguishing features of these two entities. Here, we report the first known case of bacterial peritonitis with Aeromonas hydrophilia and Escherichia coli in a patient with malignant ascites associated with pancreatic carcinoma who exhibited features of both spontaneous and secondary peritonitis. Our report suggests that clinicians should also consider bacterial peritonitis in patients with malignant ascites who present with ostensibly cancer-related symptoms.     

Multiple fibroelastomas: a case report and review of the literature.

Cardiac papillary fibroelastoma is a rare primary cardiac tumor that usually involves the heart valves. Multiple fibroelastomas found in a single patient is an even more rare occurrence. We describe the case of a 41-year-old woman who presented with an acute cerebrovascular accident, and was found to have 4 separate fibroelastoma tumors involving the aortic and mitral valves. The role of echocardiography in the diagnosis of this unusual tumor and its therapy is discussed.     

A rare giant gastric trichobezoar in a young female patient: Case report and review of the literature

A bezoar is an aggregate of undigested foreign materials that accumulate in the gastrointestinal tract and may cause serious symptoms or even life‐threatening complications. Trichobezoars, a subtype of bezoars, are a rare condition usually occurring in females with psychiatric disorders, with Rapunzel syndrome being an uncommon form of trichobezoar.     

Ganglioneuroblastoma: Case report and review of the literature

Neuroblastoma are among the most important tumors of extracranial origin in pediatric patients. They arise from embryonal cells involved in the development of the sympathetic nervous system, whose differentiation has been arrested [1,2]. They are the tumors most frequently diagnosed during the first decade of life. Their evolution is unpredictable, ranging from progression to spontaneous regression or maturation, and their location and metastatic potential vary. Little is known about the cause of these tumors and the risk factors associated with their development. This article describes a typical case of ganglioneuroblastoma and provides a review of the literature on this type of tumor.Sommario Il neuroblastoma è uno dei più importanti tumori pediatrici di derivazione extracranica; esso origina dalle cellule embrionali coinvolte nello sviluppo del sistema nervoso simpatico a causa di un blocco nel loro processo di differenziamento [1,2]. È la più frequente neoplasia della prima decade di vita; la sua progressione biologica è imprevedibile, regressione spontanea, maturazione a ganglioneuroma, localizzazione e metastatizzazione variabili. Poco è noto a riguardo dei fattori di rischio e della sua eziopatogenesi. Viene presentato un caso tipico di ganglioneuroblastoma e riesaminata la letteratura su questa neoplasia.