Available online 9 August 2008.
1. The observation
A young woman of 22 had pelvic pain without fever in December 2006. Clinical examination was normal except for left axillary lymphadenopathy. A first assessment, conducted in city found a moderate inflammatory syndrome with CRP 16 mg / l and a slight increase of CA 125. Ultrasound abdomen and pelvis showed a retro-uterine fluid blade. An abdominopelvic CT scan confirmed ascites, especially pelvic, and noted moderate homogeneous hepatomegaly. At thoracic level, there was a ganglion Lodge Baréty and some lymph infracentimétriques Aortopulmonary window. It was decided to conduct exploratory laparoscopy that found 250 ml of liquid sérohématique whose analysis showed a low cellularity with lymphocytic predominance (70%); it There were no abnormal cells; direct examination and culture were negative as well as research of BK (direct and culture). The rest of laparoscopy was normal.
That was in February 2007 and the general condition of the patient was changing. She now presented with fever peaks with abdominal pain and a weight loss of 3 kg. It was therefore achieved an excisional biopsy of left axillary lymphadenopathy. The pathologist highlighted a progressive transformation of germinal centers with nodular architecture and the presence of cells popcorn cell types evoked a paragranuloma Hodgkin nodular. Meanwhile, a positron emission tomography (PET) found foci of increased uptake and bilateral pelvic-lumbar and anterior segment V of liver. The patient then met with a hematologist for nodular lymphoma-Lennert but it was not convinced by diagnosed and addressed in internal medicine. In the service, a review led to the diagnosis and prescription of appropriate treatment. The patient was cured ...
2. The opinion of the expert-consultant
Auzary Christophe, Department of Internal Medicine, CHG de Lisieux.
This is a young woman of 22 years, which presents an array of subacute abdominal pain, accompanied by secondary fever and impaired general condition, leading to discovery, through clinical and paraclinical pelvic inflammatory disease lymphocyte and lymph node small, left axillary and mediastinal biopsy which initially evokes a progressive transformation of germinal centers (TPCG) and cells which retain the popcorn first diagnosis paragranuloma Hodgkin lymphoma nodular or nodular-Lennert. This diagnosis, refuted by a hematologist, is Adjusted internal medicine examination that leads to effective treatment that cures the patient.
2.1. A surgical emergency
First, we exclude from the outset of an emergency abdominal surgery or pelvic atypical presentation and chronic course on clinical, radiographic and laparoscopic, and scalable (ectopic pregnancy, or hydro- pyosalpinx, torsion of ovarian cyst, appendicitis, bowel perforation ...).
2.2. Malignancy
A malignancy first logically been considered: •
solid tumors, ovarian uterine, colorectal or other tumors with peritoneal carcinomatosis, could be initially feared. The high rate discreetly CA 125 is however little information contributory in the presence of ascites. The diagnosis of intra-abdominal neoplasia does not stand a laparoscopy reported normal
• lymphomatous disease: the size of the axillary lymph nodes biopsied is lacking, as is that of the lymph node of the lodge Baréty and can However, they assume were juxta-or supracentimétriques. A key element of the histological diagnosis of Hodgkin paragranuloma nodular nodular-Lennert remains quantitatively, the pathology report, the number of cells in popcorn and of course, immunohistochemistry of paragranuloma. To consider this diagnosis, it requires both large numbers of cells in popcorn and a consistent immunophenotype (CD20 +, EMA +)
. The silence is eloquent on this point: its negativity we can probably be unveiled. If
cells popcorn are few and unconvincing immunophenotype, it may include a biopsy of lymph node reaction totally nonspecific, as well as that of granuloma. Apart from a nodular lymphoma-Lennert, Hodgkin's lymphoma from another cell type, could be considered before a TPCG. Effusion lymphoma, associated with HHV8 could be discussed by Lymphocytic ascites, but consistently occurs in immunocompromised patients (hypothesis not excluded). Lymphoma seems, anyway, not be the right solution because we do not cure but complete remission.
2.3. Inflammatory diseases
Several hypotheses were plausible before histology:
• disease lupus may be responsible for lymphocytic ascites, lymphadenopathy, an enlarged liver. The existence of sterile ascites is part of the European criteria of scalability (ECLAM)
. Peritoneal fluid often contain LE cells. Abdominal inaugural presentation is classic but relatively rare (7%) and many key symptoms are lacking (skin, joints, kidney, neurological ...)
• sarcoidosis is the main granulomatosis to consider However purveyor of exceptionally serositis predominant especially in the absence of pulmonary, cutaneous, articular (one Japanese reference);
• Wegener's granulomatosis may include a utero-ovarian damage, but it lacks the lung, kidney , sinus, skin;
• in other systemic vasculitis (especially the HSP and PAN), a parietal digestion can cause peritonitis, but the clinicopathologic picture is not that which we reported;
• a autoinflammatory disease (FMF, TRAPS) can not be mentioned at this stage on a single thrust. The fever is initially lacking. The inflammation is too discreet;
• angioedema or acute porphyric do not usually ascites and suffer the same lack of recurrence
• Still disease in adults could be suggested by the association of lymph nodes, abdominal pain, but the absence of rash, arthralgia or arthritis, significant inflammation, the secondary nature of the fever are unconvincing;
• endometriosis might not have escaped the laparoscopy.
In these different conditions, marked by capricious scalability, no adequate treatment itself can not claim to cure.
2.4. Infectious diseases
This order hypothesis seems more attractive. A single treatment can cure them, many of them slow and misleading (ILT), are good purveyors of reactive lymph nodes or systemic granulomatosis [5]. Field, achievement and pelvic febrile evolution are arguments in their favor.
• TB? The negativity of cultures does not rule out this hypothesis. The chest radiograph is described (it describes only one abdominopelvic CT scan). There was no peritoneal biopsy, PCR, determination of ADA in the peritoneal fluid, or QuantiFERON IDR;
• atypical mycobacterial infection? They usually occur on land of local or systemic immunosuppression is not indicated in this observation;
• brucellosis? No element of field guides we do (work, food or travel in endemic areas). Lymph nodes, utero-ovarian impairment or ascites, however, are possible during this granulomatosis become rare in France
• Q fever? It may, in its chronic form, provide peripheral lymphadenopathy and granulomatous hepatitis but, to our knowledge, no ascites or peritoneal;
• Whipple's disease? The general picture, arthritis, digestive neuroméningé lacking. However nodal described in 52% of cases, ascites in 5%
• disease cat scratch or pasteurellosis? It could explain the lymph nodes, liver disease. Table febrile abdominal pain is more difficult to integrate: peritoneal involvement has been described other than in exceptional cases of AIDS (with bacillary angiomatosis polyserositis, pasteurellosis in a peritoneal dialysis);
• actinomycosis? Granulomatosis is possible but the peritoneal or lymph node is usually exuberant pseudotumor. It is often observed in the presence of an intrauterine device not reported here
• syphilis? That's great simulation. If hepatomegaly and polyadenopathy reaction are compatible, pelvic inflammatory disease remains difficult to integrate (literature unsuccessful);
• chlamydia? The clinical picture of abdominal pain with fever in a young woman makes a diagnosis very possible. The lymph nodes can be integrated as part of a granulomatous lymphadenitis or reactive. Lymphocytic peritonitis can
. However, no symptom of genitourinary examination or review is reported and laparoscopy is declared normal, including infringement without tubal or ovarian, without perihepatitis membranous, usually present in the syndrome of Fitz-Hugh-Curtis;
• infection virus was possible before a picture of abdominal lymph nodes with fever, including a primary HIV infection, CMV (primary peritonitis possible), EBV, herpes. Viral hepatitis B or C with cirrhosis may increase the risk of ascites, which can become infected. The count data we are deliberately concealed, but one can assume that a mononucleosis syndrome, lymphopenia or other cytopenias were absent or we would have been reported so one does not have an antigenemia and HIV serology;
• fungal infection (cryptococcosis, aspergillosis ... )? Primary peritoneal infection with lymphadenopathy is still possible but requires prior immunosuppression. Lymphadenitis, hepatomegaly can be integrated into an array of systemic fungemia but the acute course is not compatible with the table;
• parasitosis? Localized peritonitis is possible during a parasite usually affected but complicates invasive or obstructive gastrointestinal tract, especially in immunocompromised patients: during a helminth infection (strongyloidiasis, ascariasis, botriocéphalose, anisakiasis ...) or infection protozoan (leishmaniasis, amoebiasis, toxoplasmosis). Diffuse peritonitis can complicate rupture of hydatid cyst. There were no cutaneous manifestations, bilio-digestive tract usually associated with parasitosis. The pelvic ascites is not rich in eosinophils and no systemic eosinophilia is reported.
2.5. Iatrogenic disease or toxic?
granulomatosis induced by drugs would require knowledge of a prior treatment. Again the vagueness of anamnestic leaves room for the unlikely.
In extreme loneliness of the expert, at the height of the perplexity that anyone escapes, despite of fruitless and laborious attempts at rational analysis and then seek salvation in flight, it should always return to the title . But that may well be that famous Tony?
Tony Debré-Fanconi? Tony Halliday ? Tony Ugly? Tony Cardiak?
Tony Blair? This suggests that (for which the Blair) atypical Wegener's granulomatosis without involvement ENT?
Unless this is Tony Curtis? This will be our diagnostic solution, even if it seems too accessible, in a world expert believes that perhaps wrongly, from the depths of his infinite loneliness, deceptive and hostile. We therefore propose as a hypothesis No. 1 systemic infection with Chlamydia trachomatis responsible for a syndrome of Fitz-Hugh-Curtis perihepatitis not atypical. The etiological diagnosis was probably carried out by PCR on the peritoneal fluid, if not by research Chlamydia antigens in the endocervix (IF, immunoassays) by two otherwise stating seroconversion serology (serum bank).
antibiotic treatment may be associated with ceftriaxone 1 g intramuscularly to doxycycline 200 mg / d or ofloxacin 200 mg twice a day for, rather, 21 days.
3. The diagnostic approach of the authors
When she was sent into internal medicine, so this young patient had a deterioration of general condition with fever polyadenopathy may fall within the framework of a Lennert lymphoma-nodular, but mostly unexplained lymphocytic ascites lymphoma. What were the causes of ascites lymphocyte? •
diseases lymphoma, but no cases of ascites in a nodular-Lennert has so far been reported;
• carcinomatous origin seemed unlikely: no abnormality in the pelvic laparoscopy, elevated CA 125 discrete and unspecific, no suspicious cells in the ascites;
• endometritis, not view at laparoscopy;
• Tuberculosis is a common cause of ascites lymphocytic but there was little evidence for this diagnosis in our patient (IDR negative smear and culture negative peritonitis, absence of granuloma histology node);
• infection with C. trachomatis, a search of C. trachomatis by PCR had also been performed on the ascites but the result was never communicated. A call to the lab tells us that this research was positive! In re-examination
the patient, there is the notion of multiple reports of unprotected vaginal and dirty at the beginning of the unrest. Serology of C. trachomatis was positive IgA and IgG. HIV serology was negative and the VDRL-TPHA.
same time, a replay of the blades of the ganglion was performed by a specialist in hematology anatomapathologiste who finds a nonspecific reactive lymphadenitis without arguments for lymphoma. Treatment with doxycycline for three weeks is then prescribed. Three months later, the patient had no fever, and had resumed his ideal weight, there was no ascites and lymphadenopathy had regressed on the CT scan.
4. Discussion
nodular lymphoma-Lennert or Hodgkin's disease nodular lymphocyte predominance represents only 5% of Hodgkin lymphomas. It affects more men, aged between 30 and 50 years. It is manifested clinically by peripheral lymphadenopathy, often in the axilla, supraclavicular or inguinal and most often, these nodes are isolated, without any clinical sign. Diagnosis is by histological analysis of a ganglion highlighting the cells of type "popcorn" which correspond to activated B cells within germinal centers. Immunophenotyping of these cells is CD20 + CD15-CD30-. The evolution is usually favorable and requires only simple monitoring [11]. It was therefore our patient clinical and inconsistencies in particular ascites, never reported in this framework, which alerted the hematologist, thus calling into question the histological diagnosis.
C. trachomatis is the bacterial agent most frequently identified in non-gonococcal sexually transmitted infections. This Infection may take the form of urethritis, epididymitis, prostatitis a, a PID or cervicitis and sometimes ascites [12]. Ascites C. trachomatis occurs in sexually active young women often found a trigger such as a uterine manipulation or pelvic infection [10]. This ascites is usually an exudate with a high rate of protein and a component lymphocytic majority [13]. Since 1978, the role of C. trachomatis in the development of syndrome of Fitz-Hugh-Curtis is recognized [14]. The diagnosis relies on serology and appearance perihepatitis during abdominal exploration.
Our patient had a history of abortion in 2005, reports of multiple unprotected vaginal and the notion of dirty at the beginning of symptoms, which evoked a genital infection. Nevertheless, ascites C. trachomatis is infrequent, resulting in delays in diagnosis especially as the first lymph node histology oriented the diagnosis towards malignancy. Can we speak syndrome Fitz-Hugh-Curtis in this issue? One may question the existence of a hepatic uptake in PET PET study although no where perihepatitis have been reported in the literature.
In conclusion, regarding the presence of lymphocytic ascites in a woman sexually active, it must evoke and investigate infection with C. trachomatis.
Annals of Internal Medicine
Volume 29, Issue 9, September 2008, Pages 754-757 H. Maillard-Lefebvre, V. Queyrel a, M. Lambert, E. Hachulla a, D. Launay, S. Morell-Dubois, H. Charlanne a, P.-Y. Hatron a and C. Auzary b
aservice of Internal Medicine, Hôpital Claude-Huriez, CHRU Lille, rue Polonovski, 59037 Lille cedex, France
bService of Internal Medicine, CHG de Lisieux, 14100 Lisieux, France
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