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PubMed1995

[Ultrasound evaluation of acute abdominal pain in the emergency department].

In a retrospective study the value of sonography in the diagnosis of acute traumatic and nontraumatic conditions was evaluated. Records of patients who underwent emergency sonography during 1989 (group A, 159 patients) and 1991 (group B, 415 patients) were reviewed. In nontraumatic cases sonography confirmed the preliminary diagnosis in 43% and 50% of patients in groups A and B, respectively. In cases of acute abdominal pain with fever or leukocytosis, but without initial specific diagnosis, biliary disease was diagnosed in 3 group B patients; in a group A patient an abdominal aortic aneurysm was detected. There were positive sonographic findings in 42% and 17% of cases of blunt abdominal trauma in groups A and B, respectively. Sonographic evaluation of suspected splenic trauma confirmed the diagnosis in only 2 out of 9 patients in group B. Both studies show that sonography is a very effective, complementary, noninvasive method for evaluating patients with suspected acute surgical conditions. Sonography significantly reduced misdiagnosed biliary disease in cases of abdominal pain with fever or leukocytosis. In trauma, sonography obviated invasive diagnostic and surgical interventions in a significant number of cases. We recommend routine sonography in evaluating suspected, acute surgical conditions of traumatic or nontraumatic nature and in cases of undiagnosed abdominal pain with fever or leukocytosis.

Abdominal Injuries↗
PubMed1995

Treatment of experimental glioblastoma with a human major histocompatibility complex nonrestricted cytotoxic T cell line.

This study investigates a new approach to adoptive therapy of glioblastoma using as antitumor effector a potent major histocompatibility complex nonrestricted killer clone (TALL-104) established from a patient with acute T-lymphoblastic leukemia. The human glioblastoma cell line U-87 MG could be successfully engrafted in mice with severe combined immunodeficiency using the i.p., intracerebral, and s.c. routes. The latter model was elected to evaluate therapy based on its high reproducibility. Tumor growth in mice engrafted s.c. was proportionally associated with splenomegaly and leukocytosis. Multiple transfers of lethally irradiated (non-proliferating) TALL-104 cells at the tumor site resulted in about 50-70% inhibition of tumor growth as compared to untreated mice, with concomitant reduction of splenomegaly and leukocytosis. The antitumor effects were inversely proportional to the size of the tumor at initiation of therapy, 90-100% inhibition occurring in severe combined immunodeficiency mice treated from the day of U-87 MG challenge. Neither splenomegaly nor leukocytosis developed in animals in which tumor growth was completely blocked. Stimulation of TALL-104 cells with either interleukin 2 or interleukin 12 prior to irradiation and adoptive transfer increased the antitumor efficacy of the killer cells to about the same extent. The potential usefulness of irradiated TALL-104 cells in adjuvant therapy against glioblastomas and other well-localized tumors is discussed.

Animals↗
PubMed1993

The response of leukocyte subsets and plasma hormones to interval exercise.

Aerobic exercise has an established role in modulation of peripheral leukocyte concentrations. However, the effects of intense interval exercise, as employed by athletes in a range of sports, has been given little attention. Eight trained male athletes of mean age (SD) = 31.5 (4.5) yr; VO2max = 64.3 (3.8) ml.kg-1.min-1 undertook an intense interval exercise protocol (treadmill running) to exhaustion. Subjects completed an average of 15.6 1-min efforts. The protocol produced a biphasic leukocytosis: an initial (immediately posttest) leukocytosis resulting from mobilization of lymphocytes (CD3+, CD4+, CD8+, CD16+/56+, CD3+HLA/DR+) (all P < 0.01), with the later (6 h) leukocytosis resulting from mobilization of granulocytes and monocytes (both P < 0.01). This protocol modified significantly the peripheral blood concentration of the hormones cortisol (both total and free), norepinephrine, DHPG, and dopamine (all P < 0.01). Modulation of peripheral leukocyte subsets induced by interval exercise correlated with both the number of exercise efforts performed and the concomitant changes in peripheral hormone concentrations. Sustained alterations in plasma catecholamine levels in the posttest period may have important metabolic and immunological implications for athletes undertaking regular interval training.

Adult↗
PubMed1998

Hematological abnormalities and cholestatic liver disease in two patients with mevalonate kinase deficiency.

We describe two patients with mevalonate kinase deficiency and prominent hematologic abnormalities and cholestatic liver disease. Patient R.B. was not anemic at birth, but developed petechiae and cutaneous extramedullary hematopoiesis, hepatosplenomegaly, leukocytosis, and recurrent febrile events without positive bacterial or viral cultures. Patient N.M. manifested minor anomalies, hepatosplenomegaly, anemia, thrombocytopenia, recurrent febrile crises, and facial rashes. Mevalonic aciduria was found by urinary organic acid analysis, and mevalonate kinase deficiency was documented in both. The clinical spectrum of normocytic hypoplastic anemia, leukocytosis, thrombocytopenia, and abnormal blood cell forms led to diagnoses of congenital infection, myelodysplastic syndromes, or chronic leukemia in these patients before recognition of mevalonate kinase deficiency. Mevalonate kinase deficiency represents a single-gene abnormality that may be associated with significant hematologic findings. Recognition of the variability of this disorder with some patients manifesting only mild neurologic findings, yet significant hepatosplenomegaly, normocytic anemia, thrombocytopenia, and leukocytosis is important for all specialists who need to be aware of this organic aciduria.

Anemia↗
PubMed2000

Catheter-associated urinary tract infection is rarely symptomatic: a prospective study of 1,497 catheterized patients.

BACKGROUND: Catheter-associated urinary tract infection (CAUTI) is the most common nosocomial infection, accounting for more than 1 million cases each year in US hospitals and nursing homes. OBJECTIVE: To define the clinical features of CAUTI. SETTING AND PATIENTS: A university hospital; 1,497 newly catheterized patients. DESIGN: Every day that the catheter was in place, a quantitative urine culture and urine leukocyte count were obtained, and the patient was queried by a research worker regarding symptoms. To more precisely define the role of CAUTI in patients' symptoms, a subset of 1,034 patients, 89 of whom developed CAUTI with more than 10(3) colony-forming units per milliliter, who did not have another potentially confounding site of infection besides the urinary tract, was analyzed. OUTCOME MEASURES: Presence of fever, symptoms commonly associated with community-acquired urinary tract infection, and peripheral leukocytosis. RESULTS: There were 235 new cases of nosocomial CAUTI during the study period. More than 90% of the infected patients were asymptomatic; only 123 infections (52%) were detected by patients' physicians using the hospital laboratory. In the subset analysis, there were no significant differences between patients with and without CAUTI in signs or symptoms commonly associated with urinary tract infection-fever, dysuria, urgency, or flank pain-or in leukocytosis. Only 1 of the 235 episodes of CAUTI that were prospectively studied was unequivocally associated with secondary bloodstream infection. CONCLUSIONS: Whereas CAUTIs are a major reservoir of antibiotic-resistant organisms in the hospital, they are rarely symptomatic and infrequently cause bloodstream infection. Symptoms referable to the urinary tract, fever, or peripheral leukocytosis have little predictive value for the diagnosis of CAUTI.

Adult↗
PubMed2000

Clinicopathological studies of a patient with adult T-cell leukemia and pseudogynecomasty.

We present a rare case of adult T cell leukemia/lymphoma (ATL) in which leukemic T cells expressed CD4 and CD25 surface antigens and infiltrated mammary glands during clinical course of the disease. A 40-year-old male was admitted with long-standing skin lesions and leukocytosis. Peripheral blood lymphocytes were highly pleomorphic and presented CD2, CD4, CD25, CD38 membrane surface antigens. The patient proved to be seropositive for human T-cell lymphotropic virus type I (HTLV-I) antibodies. Monoclonal expansion of lymphoid cells integrated with HTLV-I genome was observed, and the diagnosis of ATL chronic type was made. He underwent a chemotherapy regimen, and skin lesions and leukocytosis improved markedly. He progressed with an indolent clinical course of ATL, when he was admitted with bilateral hyperplasia of breast, recurrent skin lesions, and leukocytosis. Breast biopsy revealed bilateral gynecomasty, extensive leukemic infiltration of typical ATL cells in the mammary glands, and the presence of mammary epithelial cells productively infected with HTLV-I. This is the first report describing invasion of the mammary tissue with HTLV-I-transformed T-cells and HTLV-I-associated breast disease.

Breast↗
PubMed1991

Clinical significance of hematologic parameters in non-Hodgkin's lymphoma at diagnosis.

Three hundred seventeen patients with non-Hodgkin's lymphoma (NHL) (54 low grade, 180 intermediate grade, 76 high grade, and seven unclassified) treated with chemotherapy were evaluated for the presence of hematologic abnormalities at diagnostic staging. Anemia was present in 42%, leukopenia in 6%, thrombocytopenia in 13%, leukocytosis in 26%, and thrombocytosis in 14% at presentation. The presence of bone marrow involvement by lymphoma was more likely to be associated with leukopenia and thrombocytopenia than the absence of bone marrow involvement. Although anemia was slightly more common in patients with bone marrow lymphoma than in those without marrow lymphoma, the difference was not statistically significant. Hematologic parameters were similar for patients with B-cell or T-cell lymphoma. Evidence of bone marrow failure with multiple cytopenias was present in 26 patients (8%). Leukoerythroblastosis was present in 2%. Circulating lymphoma was present in 9.5%. Anemic patients had a shorter survival time than nonanemic patients, whether bone marrow was involved by lymphoma or not. Survival was not affected by the presence of leukopenia or mild leukocytosis, but, in patients without marrow lymphoma, leukocytosis with a leukocyte count greater than 20 x 10(9)/l was associated with short survival length. Thrombocytopenia was associated with short survival time only in patients with bone marrow involvement by lymphoma. Patients with multiple cytopenias or leukoerythroblastosis had short survival times, but the presence of circulating lymphoma did not alter survival when compared with other patients with bone marrow involvement by lymphoma. These data suggest that hematologic evaluation at the time of diagnostic staging of NHL provides useful prognostic information that may have therapeutic implications.

Anemia, Myelophthisic↗
PubMed1985

Effects of essential fatty acid deficiency and indomethacin on histologic, ultrastructural, and phagocytic responses of hepatic macrophages to glucan.

Glucan administration in the rat induces a hyperplasia and hypertrophy of the reticuloendothelial system (RES) and a concomitant leukocytosis. Increased phagocytic function and lysozymal immunoreactivity of macrophages are also characteristic of the glucan effect. The potential role of arachidonic acid metabolites in mediating this hepatic inflammatory response induced by the RES stimulant glucan was assessed in the present study by two experimental approaches. In one study, rats were depleted of arachidonic acid by rendering them deficient in essential fatty acids (EFA). In another study, rats were pretreated with the fatty acid cyclooxygenase inhibitor indomethacin. Both treatment interventions markedly attenuated the hepatic Kupffer cell proliferative and granulomatous response to glucan and the associated leukocytosis. Lysozyme immunoreactivity of the Kupffer cells and rates of colloidal carbon clearance (T/2), however, were enhanced by the above treatments. Supplementation of EFA-deficient rats with ethyl arachidonate restored their glucan response to an extent that was not significantly different from nondeficient rats. Marked hepatic proliferative responses were apparent only in those treatment groups characterized by leukocytosis, which suggests that extrahepatic recruitment is an important component of the glucan response in normal, nutritionally adequate rats. Collectively these data suggest that arachidonic acid metabolites may play a role in modulating this extrahepatic recruitment and the associated cellular proliferative and granulomatous responses following glucan administration of the rat.

Animals↗
PubMed1999

Influence of incisional complications on infrainguinal vein bypass graft outcome.

Incisional complications following infrainguinal vein bypass remain a formidable challenge to the vascular surgeon. A retrospective analysis of 250 infrainguinal vein bypass grafts (214 patients) was undertaken to identify risk factors for the development of incisional complications and determine the impact of incisional complications on outcome. Incisional complications occurred in 47 (19%) of the bypasses. The mean age was 67.8 years and 155 (62%) of the bypasses were performed in men. The patients were divided into two groups based on the presence or absence of an incisional complication. By univariate analysis, the contributing factors for an incisional complication were: veteran hospital status, dialysis dependence, anemia, obesity, leukocytosis, and hypoalbuminemia (p < 0.05). By multivariate analysis, veteran hospital status, female gender, leukocytosis, and prior ipsilateral bypass were independent risk factors for incisional complications (p < 0.05). The increased incidence of incisional complications in the veteran's hospital group was associated with anemia, leukocytosis, decreased lymphocyte count, and increased incidence of tissue loss (p < 0.05). By life-table analysis, incisional complications did not influence primary patency (p = 0.73), secondary patency (p = 0.91), limb salvage (p = 0.69), or survival (p = 0.92). However, a significant suppurative soft-tissue infection ultimately resulted in a high rate of major amputation.

Aged↗
PubMed1987

Interactions between local inflammatory and systemic haematological effects of PAF-acether in the rat.

Intraplantar injections of PAF-acether into the rat paw (0.025-16 micrograms/paw) were followed by a bell-shaped dose-response curve for oedema. In parallel to the ascending phase (0.25-2 micrograms/paw) a significant leukopenia was observed whereas thrombocytopenia, haemoconcentration and leukocytosis were seen during the descending phase (4-16 micrograms/paw) as was also observed after the i.v. injection of PAF-acether. I.v. injections of PAF-acether inhibited dose dependently the oedema induced by PAF-acether itself but were inactive against serotonin. Dexamethasone (0.1 mg/kg) inhibited the PAF-acether-induced thrombocytopenia and haemoconcentration but failed to modify the leukocytosis whereas aspirin (100 mg/kg) and indomethacin (1.0 mg/kg) inhibited only the haemoconcentration. The PAF-acether antagonist 48740 RP blocked the systemic haematological alterations and restored the auto-reduced oedema induced by a high dose of PAF-acether (16 micrograms/paw) but was unable to inhibit the paw oedema induced by PAF-acether. It is suggested that haemoconcentration, thrombocytopenia and leukocytosis are independent phenomena associated with the presence of PAF-acether in the bloodstream, these haematological changes and the local oedema induced by PAF-acether involve distinct mechanisms and the auto-inhibitory property of PAF-acether is not restricted to in vitro situations, but extends to local inflammation.

Animals↗
PubMed1996

Mixed myelodysplastic and myeloproliferative syndromes.

Aplastic anemia, myelodysplastic syndromes (MDS) and chronic myeloproliferative diseases (MPD) are stem cell disorders. There is no clear-cut demarcation of them. Hypoplastic MDS displays features of aplastic anemia and MDS, on the other side mixed myelodysplastic and myeloproliferative syndromes (MDS-MPS) develop. In our collection of 566 MDS patients, features of myelodysplasia as well as myeloproliferation, MDS-MPS, were present in 25 patients (4.4%). Twelve patients had at the time of diagnosis megakaryocytic proliferation and thrombocythemia beside signs of MDS, and seven had myelodysplasia with granulocytic proliferation and leukocytosis. In another six patients, MDS was the first diagnosis and the proliferative phase developed later during the course of the disease. These patients can be characterized as MDS-MPS in evolution. All subjects had a variable degree of anemia. While the level of thrombocythemia has been relatively stable, the number of leukocytes has been progressive, but rarely extended beyond 100 x 10(9)/l. Ring-sideroblasts and myelofibrosis were frequent findings. Two more homogeneous MDS-MPS groups emerged in our analysis: sideroblastic anemia with thrombocythemia and a group fulfilling the criteria of Philadelphia chromosome negative and bcr-abl negative "atypical chronic myeloid leukemia (aCML)'. One patient with thrombocythemia and three with leukocytosis (23%) transformed to acute myeloid leukemia (AML). Men prevailed (12/13) in patients with leukocytosis and MDS-MPS in evolution. Of the 46% MDS-MPS patients with chromosomal aberrations, del(20)(q) is of interest.

Adult↗
PubMed1986

The role of complement in inflammation during experimental pneumococcal meningitis.

The mechanism whereby an effective bactericidal inflammatory reaction develops in the subarachnoid space is not clearly defined. While normal cerebrospinal fluid is deficient in complement, immunoglobulin and leukocytes, these serum components appear in cerebrospinal fluid (CSF) during the course of bacterial meningitis. Using a rabbit model of pneumococcal meningitis we examined the role of the alternate complement pathway in three early events important to the defense of the subarachnoid space: leukocyte chemotaxis, phagocyte mediated bacterial killing, and clearance of bacterial components from the cerebrospinal fluid space. Rabbits treated with cobra venom factor to deplete complement were inoculated intracisternally with encapsulated (type II or XIX) pneumococci. Following complement depletion, there was a dramatic (at least 100-fold) decrease in the LD50 for these strains. Nevertheless, complement depletion did not affect the magnitude of CSF leucocytosis or the rate of clearance of bacterial particles from CSF. A short delay in the appearance of leukocytes in CSF was found in the absence of complement. The major effect of complement depletion, however, was to diminish the efficiency of leukocyte mediated killing of encapsulated bacteria in the CSF. Although the short delay in the onset of leukocytosis in the complement depleted animals is consistent with a chemotactic role of complement in the normal animal, the quantitatively normal leukocytosis in the complement depleted rabbits clearly indicates that important chemotaxins other than complement function in CSF. Inhibition of leukocytosis by indomethacin and diclofenac suggests that metabolite(s) of the arachidonic acid pathway may perform such a chemotactic role. A major role of complement in the defense of the subarachnoid space appears to be as an opsonin needed for the effective bactericidal activity of leukocytes. It is the lack of this function that best explains the greatly decreased LD50 value of encapsulated pneumococci in the complement depleted animal.

Animals↗
PubMed2004

Determinants of severity for superficial cellutitis (erysipelas) of the leg: a retrospective study.

BACKGROUND: Superficial cellulitis (erysipelas) of the leg is a frequent infectious disease with a favorable outcome, whereas some patients present a serious disease. The determinants of severity for superficial cellulitis (erysipelas) of the leg have not yet been clearly established. In order to determine the characteristics of patients presenting with severe superficial cellulitis of the leg, we analyzed patients with favorable and unfavorable outcome. METHODS: The records of 167 patients referred to Rouen University Hospital for non-superficial cellulitis of the leg were analyzed. Two severity groups of patients were retrospectively defined. Patients in the severe group either died secondary to infection during hospital stay or were hospitalized for a duration at least equal to the 90th percentile (i.e., >21 days of hospitalization). The remaining patients were considered as presenting with non-severe cellulitis. Potential determinants of severity were analyzed by univariate and multivariate analysis based on logistic regression. RESULTS: From univariate analysis, the following general factors were positively associated with severity: advanced age, arterial hypertension, diabetes mellitus, elevated leukocytosis, and elevated neutrophilia. The local factors associated with severity were ulcer of the leg and arteriosclerosis obliterans of the leg. From multivariate analysis, only age (P=0.004), diabetes mellitus (P=0.01), and leukocytosis (P=0.04) appeared to be independently associated with severity. A close to significant association was also found with arteriosclerosis obliterans of the leg (P=0.07). Whereas general complications occurred more frequently in the severe group, no such difference was observed for local complications. CONCLUSIONS: Determinants of severity for superficial cellulitis of the leg include high age and associated medical conditions. Aged patients and patients with diabetes mellitus, elevated leukocytosis, or possibly arteriosclerosis obliterans of the leg should preferably be hospitalized for specific care of associated conditions to avoid the occurrence of general complications.

Journal Article↗
PubMed2006

Alcoholic hepatitis with leukemoid reaction after surgery.

Alcoholic hepatitis (AH) is a clinicopathologic syndrome resulting from an excessive intake of alcohol. Leukemoid reactions (LRs) are characterized by a strikingly elevated granulocyte count over 40,000-50,000 cells/mm(3). Although a leukocytosis of 15,000-18,000 cells/mm(3) is frequently seen in AH, LRs are rare in this context. AH-associated LRs are a sign of poor prognosis and have a high mortality. A 64-year-old male with a history of heavy alcohol intake underwent a right hemicolectomy for cecal carcinoma. Preoperative laboratory data were normal with the exception of an albumin of 2.1 g/dL. Liver biopsies that were taken because of a nodular appearance revealed micronodular cirrhosis, steatohepatitis, and Mallory bodies. Postoperatively, the patient developed a leukocytosis that progressively increased to 72.6 cells/mm(3). He also developed signs of impaired hepatic and renal function. Extensive workup failed to reveal a source of infection. A trial of intravenous antibiotics had no impact on the leukocytosis. Methylprednisolone at a dose of 40 mg IV daily was started on postoperative day 9. The patient experienced a progressive decline in white blood count (WBC), which reached 25.2/mm(3) on postoperative day 14. However, he died on postoperative day 16. We conclude that the patient had AH-associated LR in the postoperative period, but died despite successful treatment of the LR with steroids.

Carcinoma↗
PubMed2005

Pediatric parotitis: a 5-year review at a tertiary care pediatric institution.

BACKGROUND: Parotitis is a well recognized entity in the adult population, however there are very few studies concerning the clinical presentation and management of this condition in children. OBJECTIVES: To characterize pediatric parotitis in a tertiary care setting, with the goal of clarifying management recommendations and outcomes. METHODS: The charts of all pediatric patients with a diagnosis of parotitis treated at a tertiary care academic institution from 1999 to 2004 were reviewed. The management of inpatients and outpatients were characterized to define differences in presentation and care. RESULTS: Twenty-one children (6 months-15 years) with a diagnosis of parotitis were identified. Thirteen (62%) children were treated as inpatients, of which seven (54%), had significant medical co-morbidities. The most common clinical presentations of the inpatient group included dehydration (46%), fever (38%) and leukocytosis (46%). Two inpatients (15%) required surgical drainage due to abscess formation. Eight children (38%) were treated as outpatients, none with associated co-morbidity, fever, leukocytosis, or complication due to infection. All outpatients were treated with oral antibiotics or conservative therapy with eventual resolution. CONCLUSIONS: Parotitis in the pediatric population is uncommon. The presence of a significant co-morbidity, fever, or leukocytosis may require inpatient therapy and imaging if patients fail to improve with medical therapy. Other than abscess drainage, surgery for parotitis in children is not routinely recommended.

Abscess↗
PubMed1997

Uteroferrin and recombinant bovine GM-CSF modulate the myelosuppressive effects of 5-fluorouracil in young female pigs (Sus scrofa).

The present study investigated the ability of uteroferrin and recombinant bovine granulocyte monocyte/macrophage-colony stimulating factor (rbGM-CSF) to modulate the myelosuppressive effects of 5-fluorouracil (5-FU) in young female pigs (Sus scrofa). Pigs (N = 3/treatment) were infused with 5-FU (32.5 mg/kg) on days 0 and 1 of the experimental period. Uteroferrin (100 micrograms/kg in 0.9% NaCl), rbGM-CSF (10 micrograms/kg in 0.9% NaCl), uteroferrin + rbGM-CSF (as above) or control (0.9% NaCl) were administered as intramuscular injections twice daily (0800 and 2000 hr). Peripheral blood cell number, composition, and progenitor cells were determined over 28 days. Treatment of pigs with 5-FU resulted in a rapid leukocytopenia and thrombocytopenia (nadirs on days 5 and 7, respectively) and a modest decrease (P < 0.05) in red blood cell (RBC) number (nadir on day 14). Although nor affecting RBC and thrombocytes, treatment of pigs with uteroferrin had an initial protective effect (P < 0.05) on the 5-FU-induced leukocytopenia (63 and 64 vs 48 and 39 +/- 6% of baseline on days 3 and 5, respectively). In contrast, rbGM-CSF enhanced (P < 0.05) the rate of the leukocytopenia and had only minor effects on thrombocyte numbers relative to controls. These effects appeared to be additive, as pigs treated with uteroferrin + rbGM-CSF had a reduced rate of leukocytopenia compared to pigs treated with rbGM-CSF alone. Uteroferrin + rbGM-CSF also attenuated (P < 0.05) the suppression and enhanced (P < 0.05) recovery of RBC and thrombocyte numbers following 5-FU treatment. In control pigs, a modest rebound leukocytosis (122 +/- 6% of baseline) and thrombocytosis (141 +/- 9% of baseline) was evident. Uteroferrin enhanced (P < 0.05) the rebound leukocytosis (135 +/- 6% of baseline), but attenuated (P < 0.05) the thrombocytosis. In contrast, rbGM-CSF enhanced (P < 0.05) the duration of the leukocytosis during the recovery phase, an effect augmented by the combination of uteroferrin + rbGM-CSF. In addition, treatment with uteroferrin + rbGM-CSF resulted in a sustained thrombocytosis (days 12 to 21). As indicated by changes in CFU-GM, BFU-E, and CFU-GEMM progenitor cells in peripheral blood, the effects of uteroferrin and rbGM-CSF appeared to reflect their ability to enhance the proliferation and/or differentiation of both similar and distinct hematopoietic progenitor cells.

Acid Phosphatase↗
PubMed1991

Adult-onset Still's disease.

Adult onset Still's disease seems to be the adult form of Still's disease in children. The key symptoms of the disease are high spiking fever, arthritis and a macular or maculopapular, salmon-pink evanescent rash, almost always accompanied by neutrophilic leukocytosis and frequently by sore throat, intense myalgias, lymphadenopathy, splenomegaly and signs of serositis. Tests for IgM rheumatoid factor and antinuclear antibody are characteristically negative. With respect to haematologic abnormalities, the disease may give rise to several problems. First, there is a neutrophilic leukocytosis, which currently is unexplained, and often a normocytic normochromic anaemia, that may be profound. The anaemia has the characteristics of anaemia of chronic inflammatory disease. Both abnormalities disappear after effective treatment of the disease or at spontaneous remission. Secondly, there might be a problem to differentiate AOSD from malignant haematological disorders, including malignant lymphoma and leukaemia, especially when the picture is dominated by lymphadenopathy, splenomegaly, fever and leukocytosis. Although in rare cases the differential diagnosis is extremely difficult, diagnosis can mostly be made or excluded by peripheral blood smear staining, bone marrow biopsies and occasionally lymph node biopsy. Finally, like the juvenile counterpart, AOSD is occasionally complicated by sometimes life-threatening diffuse intravascular coagulation. Factors that might be important in the development of this complication include severe disease activity, liver abnormalities and particular drugs including salicylates, other NSAIDs and some slow-acting antirheumatic drugs. Prompt therapy, including withdrawal of the drug, corticosteroids and sometimes anticoagulant therapy have been successfully applied to most patients.

Adrenal Cortex Hormones↗
PubMed2001

Chronic neutrophilic leukemia (CNL): a clinical, pathologic and cytogenetic study.

This report describes a single institution's recent experience with six patients fulfilling the diagnostic criteria of chronic neutrophilic leukemia. No patient had the Philadelphia chromosome or the BCR/ABL fusion gene. None of the common cytogenetic abnormalities characteristic of myeloid disorders were detected. Two patients demonstrated clonal evolution during the course of the disease. All responded initially to therapy with hydroxyurea with control of leukocytosis and reduction in splenomegaly. Three patients eventually became refractory to hydroxyurea, manifesting progressive neutrophilia without blastic transformation. Aggressive chemotherapy to control progressive leukocytosis resulted in death due to cytopenias in two of these patients. The third patient received less intensive chemotherapy and died of progressive disease. One patient died after transformation of the disease into undifferentiated acute myeloid leukemia. Two patients remain alive with stable disease on hydroxyurea therapy, 12 and 54 months after initial diagnosis. Chronic neutrophilic leukemia is a rare clinicopathologic entity that can be distinguished from chronic myelogenous leukemia, the recently described neutrophilic-chronic myelogenous leukemia, and myelodysplastic syndrome. The clinical course is heterogeneous, with a definite risk of death from either blastic transformation or progressive neutrophilic leukocytosis. Continued study and reporting of these cases must be encouraged.

Humans↗