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PubMed2000

Platelet production in the pulmonary capillary bed: new ultrastructural evidence for an old concept.

Although there is substantial evidence indicating that platelets are released from megakaryocytes in the capillary bed of the lung, this concept has not been universally accepted because much of the evidence has been indirect. To more definitively substantiate that platelet production takes place in the lungs, megakaryocyte and platelet production was accelerated in mice by phlebotomy or by administration of thrombopoietin, and ultrastructural analysis was performed on lung specimens. Intact megakaryocytes, megakaryocyte fragments with numerous demarcated platelet fields, dissociating intact platelets, and denuded megakaryocyte nuclei were seen in the pulmonary capillaries of mice. In addition, some megakaryocyte nuclei exhibited the morphological counterpart of apoptosis. These observations provide evidence for platelet release in the capillary bed of the lungs during stimulated as well as reactive thrombocytosis without precluding observations that some "proplatelets" form in the sinusoids of the bone marrow before transmigration of intact megakaryocytes into the circulation.

Animals↗

PubMed2003

Functions of the splenic remnant after subtotal splenectomy for treatment of severe splenic injuries.

BACKGROUND: To evaluate the clinical and laboratory characteristics of patients submitted to subtotal splenectomy during the immediate and late postoperative period. METHODS: The study was conducted on 34 patients, 25 of whom were submitted to subtotal splenectomy (group I), and 9 to total splenectomy without preservation of splenic tissue (group II), and on 22 patients with intact spleens (group III, control). The immediate and late postoperative complications were investigated. Hematological examinations were performed during the late postoperative period (red cell count, hemoglobin, platelets, total and segmented leukocytes, lymphocytes, and Howell-Jolly bodies). Immunoglobulins (IgA, IgM, and IgG) and total T lymphocytes (TTL), active T lymphocytes (ATL), and B lymphocytes were also determined. Splenic scintigraphy with (99m)Tc colloidal sulfur was performed. RESULTS: Groups I and III did not presented abnormal blood bodies and their hematological and immunological pattern were normal. None of the groups showed leukocytosis or thrombocytosis. Howell-Jolly bodies were observed only in group II, which also showed reduced IgM levels. Scintigraphy showed filtering splenic tissue in group I. CONCLUSIONS: We conclude that subtotal splenectomy is a good surgical alternative for serious distal spleen lesion or when the main splenic pedicle is injured.

Adolescent↗

PubMed1996

Hematology for primary care physicians.

Many hematologic disorders present minimal physical signs and symptoms in the early state. For example, chronic myelogenous leukemia may not manifest splenomegaly or any obvious physical signs, yet the laboratory report may demonstrate leukocytosis, eosinophilia, basophilia, and thrombocytosis. Although the anemic condition of a patient with a hemoglobin level of 7 gm/dl may be readily apparent to the clinician, a hemoglobin level of 10.5 gm/dl may be difficult to discern during a brief visit that is focused on another organ system. The same laboratory report, however, may contain valuable clues about unsuspected anemia related to mean corpuscular volume or morphology. Information from supporting chemistry studies often may be helpful in interpretation of the diagnosis. An elevated uric acid level, for example, may indicate hyperkinetic cytogenesis related to myeloproliferative or lymphoproliferative neoplastic disorders. This monograph is designed to be useful to busy physicians who want to use basic hematologic studies in a cost-effective manner. Hematology is viewed in a problem-oriented way; the laboratory report is used as the problem generator.

Anemia↗

PubMed1996

Abdominal inflammatory myofibroblastic tumors in children: report of an appendiceal case and review of the literature.

Inflammatory myofibroblastic tumors (IMT) are rare, benign lesions. They mimic, clinically and radiologically, malignant tumors-especially sarcoma. IMT most often are seen in the lung of young adults, but there have been reports of these tumors occurring in children, in various sites. The authors report a case of appendiceal IMT arising in a 15-year-old boy previously treated for inherited distal renal tubular acidosis. He was admitted because of anorexia, weight loss, and a low-grade fever. The ultrasound study showed bilateral hydroureteronephrosis, with a poorly functioning right kidney noted on intravenous urography, and a retrovesical soft-tissue mass. Laboratory studies showed renal insufficiency, an elevated white blood cell count, anemia, thrombocytosis, and hypergammaglobulinemia. During laparotomy, a pelvic appendiceal mass was discovered, appendectomy was performed, and the pathological diagnosis of inflammatory myofibroblastic tumor was made. Three months later the boy was well, and the biological, sonographic, and urographic results had returned to normal. A review of 74 cases of abdominal IMT occurring in childhood emphasizes the importance of pathological differentiation of these lesions from malignancy, with early total excision whenever possible. Long-term follow-up is necessary to detect local recurrence, which may develop many years later, and to confirm the allegedly benign nature of these tumors.

Adolescent↗

PubMed1990

Effects of recombinant human erythropoietin in infants with the anemia of prematurity: a pilot study.

In an attempt to stimulate endogenous erythrocyte production and thereby provide an alternative to erythrocyte transfusions, we administered recombinant human erythropoietin (rHuEpo) in doses of 75 to 300 units/kg/wk to seven infants with the anemia of prematurity. Treatment was started between 21 and 33 days of life, maintained for 4 weeks, and was well tolerated. All the patients had low baseline serum erythropoietin levels. After rHuEpo therapy, the number of reticulocytes increased from a mean baseline count of 75 x 10(9)/L to 95, 141, and 165 x 10(9)/L on days 7, 10, and 14 of therapy, respectively. Correction or stabilization of the anemia was observed in six of seven patients, whose estimated total erythrocyte volume increased by 49% during therapy (vs a predicted increment of 18% in the absence of rHuEpo). In one patient, however, the hematocrit declined during the treatment, and in three of the responders a secondary fall in hematocrit was noted either during therapy or after its discontinuation. Serum iron and ferritin levels rapidly decreased after the initiation of rHuEpo therapy, and in most patients transient early thrombocytosis and late neutropenia were observed. These data suggest that rHuEpo may correct or stabilize the anemia of prematurity. Its effects, however, may be limited by a variety of factors, among which iron availability probably plays an important role. Controlled studies will be needed to confirm these preliminary observations.

Anemia↗

PubMed1978

Peripheral total parenteral nutrition for premature infants with the respiratory distress syndrome: a controlled study.

In 40 premature infants, in whom severe respiratory distress precluded oral feeding, peripheral total parenteral nutrition consisting of casein hydrosylate, dextrose, and soybean emulsion was compared to nutrition with dextrose and electrolytes. The TPN group received more calories, and the total serum protein in them increased significantly. Metabolic complications did not occur. Transient thrombocytosis occurred in six infants who received TPN and eosinophilia occurred in nine. The case fatality rate in the TPN group (three of 20) was not significantly less than in the glucose group (six of 20). The differences suggested that infants who weigh less than 1,500 gm may receive the greater benefit from TPN.

Blood Proteins↗

PubMed1984

Prospective comparative trial of moxalactam versus ampicillin or chloramphenicol for treatment of Haemophilus influenzae type b meningitis in children.

In a prospective, randomized study, moxalactam in 44 children was compared with ampicillin or chloramphenicol in 47 children for the treatment of Haemophilus influenzae type b meningitis. Both groups were comparable in terms of clinical and laboratory findings at admission. The hospital course, neurologic sequelae including deafness, and number of deaths were the same for both groups. The incidence of adverse reactions also was the same except that diarrhea and thrombocytosis occurred significantly (P less than or equal to 0.04) more frequently in children given moxalactam. Moxalactam was equivalent to ampicillin or chloramphenicol in the treatment of H. influenzae type b meningitis in children.

Ampicillin↗

PubMed1999

The extended oligoarticular subtype is the best predictor of methotrexate efficacy in juvenile idiopathic arthritis.

OBJECTIVE: To determine whether demographic, clinical, and laboratory variables measurable at baseline predict the clinical efficacy or major toxic effects of methotrexate (MTX) therapy in children with chronic arthritis. STUDY DESIGN: Patient eligibility criteria: (1) monitored in our unit between 1986 and 1996 with a diagnosis of chronic arthritis and (2) treatment with MTX as the sole second-line agent and for at least 6 months. Outcomes investigated: (1) short-term (6-month) clinical response, (2) complete disease control, (3) disease relapse after MTX discontinuation after complete disease control, (4) aminotransferase elevation, (5) gastrointestinal toxicity. Independent variables that showed significant results with univariate tests or were clinically relevant for each outcome underwent multiple logistic or Poisson regression analyses. RESULTS: Eighty patients were available for analysis. The disease onset subtype was systemic in 37 patients, polyarticular in 20 patients, and oligoarticular in 23 patients (all with polyarticular course: extended oligoarticular subtype). The extended oligoarticular subtype was the best predictor for both the short-term clinical response (odds ratio 6.80, P =.02) and, together with a better functional ability, the complete disease control (rate ratio 3.85, P =.03 and rate ratio 3.29, P =.006, respectively). Patients with this subtype of chronic arthritis tended to have earlier, and more frequently, a disease relapse after MTX discontinuation. Thrombocytosis was the only significant risk factor for liver biochemical abnormalities (rate ratio 2.94, P =.008), whereas no variable yielded significant results for gastrointestinal toxicity. CONCLUSION: Patients with extended oligoarticular chronic arthritis were more likely to benefit from MTX therapy and to have a relapse after treatment discontinuation, suggesting that MTX is distinctly more effective in this subset of chronic arthritis.

Adolescent↗

PubMed1986

Leukemoid reaction in a patient with bladder and prostatic cancer.

Leukemoid reactions often are seen in patients with underlying malignancies but they have been reported infrequently in patients with urological malignancies. We report reactive leukocytosis and thrombocytosis in a patient with bladder and prostatic carcinoma. Both hematological abnormalities resolved with definitive surgical therapy. We also review other reported cases of leukemoid reactions in patients with urological malignancies.

Adenocarcinoma↗

PubMed1986

Myeloid metaplasia masquerading as a urethral caruncle.

A 64-year-old woman presented with a painless bleeding mass of the urethral meatus that was diagnosed as a caruncle and removed surgically. Histological examination revealed a cellular and vascular polypoid lesion that contained megakaryocytes, as well as groups of hematopoietic stem cells, and maturing cells of the erythroid and granulocytic series. The diagnosis of myeloid metaplasia was confirmed by marker studies with immunoperoxidase techniques. The patient had a long history of bleeding and clotting problems owing to a myeloproliferative disorder with thrombocytosis and gradual development of myelofibrosis. No other foci of extramedullary hematopoiesis were apparent. The patient was free of urinary symptoms 22 months postoperatively.

Diagnosis, Differential↗

PubMed1991

Essential thrombocythemia in young adults.

Essential thrombocythemia is typically a disorder of adults in the sixth or seventh decade of life and is characterized by frequent thrombohemorrhagic complications. In young patients, the optimal management of complications is controversial. We studied 56 young adults (33 female and 23 male patients) with a diagnosis of essential thrombocythemia. The mean duration of follow-up was 4.68 years. The mean platelet count at diagnosis was 1,328,000/mm3. Platelet aggregation studies in 21 patients demonstrated hypoaggregation to epinephrine; spontaneous platelet aggregation was present in 4. At diagnosis, 39 patients were asymptomatic, and thrombocytosis was discovered incidentally. Throughout follow-up (up to 20 years), 24 patients remained asymptomatic. Thrombotic complications developed in 24 patients; they were life-threatening in only 3. The most common vaso-occlusive symptoms were migraine headache (in 12 patients) and erythromelalgia (in 3). Minor hemorrhagic complications occurred in six patients, and none was life-threatening. Serious complications (one cerebral and two myocardial infarctions) occurred in three patients, all of whom recovered. Two deaths occurred, neither of which was attributable to essential thrombocythemia. The treatment regimens used were chemotherapy in 9 patients, antiaggregating agents in 7, radioactive phosphorus in 1, the newer platelet-lowering agent anagrelide in 10, and only observation in 29. No treatment-related acute leukemias developed. This series of young patients with essential thrombocythemia, the largest to date, demonstrates a low incidence of life-threatening complications and a favorable long-term prognosis. Therapeutic recommendations should remain conservative, and potential leukemogens should be avoided unless serious complications develop. Anagrelide may be useful in young patients with thrombocythemia who are symptomatic.

Adolescent↗

PubMed1986

Cogan's syndrome: 18 cases and a review of the literature.

In this article, we review 78 cases of Cogan's syndrome--18 from our institution and 60 from the English medical literature--and describe the clinical, laboratory, radiographic, and pathologic findings associated with this disorder. Patients with Cogan's syndrome should be examined by an ophthalmologist, otolaryngologist, and internist because, in addition to the eye and audiovestibular involvement, systemic manifestations are common. An elevated erythrocyte sedimentation rate, anemia, leukocytosis, and thrombocytosis are common but nonspecific laboratory abnormalities. Serious outcomes include deafness and, less frequently, vasculitis, aortic insufficiency, blindness, and death. Glucocorticoids seem to be effective therapy for active Cogan's syndrome. Aortic valve replacement and vascular bypass grafting are indicated in selected cases. The roles of cytotoxic-immunosuppressive agents and cochlear implantation remain to be clarified.

Adolescent↗

PubMed1986

Vascular complications of inflammatory bowel disease.

During an 11-year period from January 1970 to December 1980, 7,199 patients at our institution had chronic ulcerative colitis or Crohn's disease. Thromboembolic complications developed in 92 (1.3%) of these patients. An additional 4 patients had cutaneous vasculitis, and 17 had an arteritis-associated diagnosis. Of the thromboembolic complications, 61 were deep vein thromboses or pulmonary emboli. The mortality among patients with thromboembolic complications was high (25%). Sixty percent of the patients had a thrombocytosis unaffected by sulfasalazine or corticosteroid therapy. In 73% of the patients, the erythrocyte sedimentation rate was increased, and when measured, fibrinogen and factor VIII were commonly elevated. Peripheral arterial thrombosis, coronary thrombosis, and mesenteric and portal vein thrombosis were predominantly postsurgical complications, but 77% of peripheral venous thromboses occurred spontaneously. The role of anticoagulation and surgical intervention in the management of hypercoagulation in patients with inflammatory bowel disease is discussed.

Adolescent↗

PubMed1988

Malignant mesothelioma of the pleura.

Malignant mesothelioma of the pleura most commonly occurs in persons with a heavy occupational exposure to asbestos. Some patients, however, have no such history of exposure. Clinical features include initial complaints of nonpleuritic chest pain and dyspnea. The most frequent roentgenographic finding is a unilateral pleural effusion. Thrombocytosis and elevated erythrocyte sedimentation rates are common. Pleural effusions are typically exudates, are often hemorrhagic, and are usually insufficient for diagnosing mesothelioma based on cytology alone. Even pleural biopsy may not produce enough tissue to enable the pathologist to make a firm diagnosis. Thoracotomy and open biopsy will confirm the diagnosis in most cases. Pathologic distinction from metastatic adenocarcinoma may be difficult even after the use of special stains and electron microscopy. Clinical deterioration is primarily attributable to local spread of tumor. Several factors seem to predict prolonged survival: (1) epithelial histologic subtype, (2) performance score, (3) age of the patient at the time of diagnosis, and (4) absence of chest pain. Surgical treatment, chemotherapy, and irradiation, alone or in combination, have been used for malignant mesothelioma. Except for the palliative effect of irradiation, most treatment protocols have not altered the dismal median survival of approximately 11 months seen in untreated patients with malignant mesothelioma.

Adenocarcinoma↗

PubMed1979

Anticoagulant treatment to prevent cerebral infarction.

The indications for anticoagulant treatment to prevent cerebral infarction or progression of cerebral infarction are now clear. The indications are: (1) Prevention of recurrent embolization from a cardiac source (long-term anticoaguland treatment). (2) Transient ischemic attacks (particularly vertebrobasilar system) if a surgically accessible causative lesion, polycythemia, and thrombocytosis are not present (anticoagulants for a few months.) (3) Progressing stroke in either systme assuming that the neurological defect is partial and CT scan shows no evidence of bleeding (anticoagulants for a few months.) (4) Rarely, completed stroke (long-term).

Anticoagulants↗

PubMed2004

[POEMS syndrome (or Crow-Fukase syndrome)].

POEMS is an acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. POEMS syndrome is also called Crow-Fukase syndrome, chiefly in Japan. The 5 above mentioned features are not always present at the first examination. The minimal criteria to establish the diagnosis are the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammopathy, the light chain being almost always lambda, and at least 2 of the 8 other features: sclerosing plasmocytoma, endocrinopathy, skin changes, organomegaly, Castleman's disease, anasarca, papillary edema or thrombocytosis. Among these features, only cutaneous glomeruloid angioma are specific. Ultrastructural identification of uncompacted myelin lamellae on the peripheral nerve biopsy is also a strong argument in favor of the diagnosis. An associated "osteosclerotic" bone lesion must be carefully searched, because its treatment may improve the other features of the syndrome, especially the neuropathy. Cytokines and the vascular growth endothelial factor might play a role in the pathogenesis of this rare multisystemic disorder.

Humans↗

PubMed2004

[Cerebral infarction of arterial origin and haematological causation: the Lausanne experience and a review of the literature].

INTRODUCTION: Hematological diseases are seldom found as the etiology of ischemic strokes, but are frequently investigated by expensive laboratory tests after a first cerebral vascular event. METHODS: In the Lausanne Stroke Registry, we retrospectively reviewed the cases of patients hospitalized between 1979 and 2001 for a first ischemic arterial stroke which was attributed to a hematological etiology. Of 4697 patients, 22 (0.47 per cent) had a stroke due to one of the following hematological pathology: polycythemia vera (4), secondary polycythemia (4), essential thrombocytemia (2), secondary thrombocytosis (4), multiple myeloma (1), CIVD (1), protein S deficiency (1), antiphospholipid antibody syndrome (4), moderate homocysteinemia (1). A literature review was undertaken for each hemopathy. CONCLUSION: In light of the results of these data, we concluded that a complete blood count provides sufficient hematological screening for the majority of patients hospitalized for an arterial stroke. The antiphospholipid antibody syndrome is a rare cause of cerebral infarction, which needs to be investigated in young patients, in cases of multiple or recurring stroke or in the presence of a typical history. Inherited thrombophilias are not a significant risk factor for arterial cerebral infarction and their investigation is only warranted for a sub-group of young patients with a cryptogenic stroke, in which group the prevalence is slightly increased. Moderate homocysteinemia must be considered as a cerebrovascular risk factor of minor importance, but potentially treatable by a substitution of vitamin B12, B6 and folates. The efficacy of this substitution in the prevention of cardiovascular events needs yet to be demonstrated.

Adult↗

PubMed2001

Chronic myeloid disorders: Classification and treatment overview.

Chronic myeloid disorders (CMD) are collectively characterized by monoclonal myeloproliferation that involves multiple lineages, retains a variable degree of cellular maturation, and has the potential to undergo clonal evolution. However, monoclonal hematopoiesis is neither essential nor specific to CMD. Morphologic and cytogenetic characteristics allow a working classification of these disorders that is clinically useful. There are four major divisions: chronic myeloid leukemia (CML), which is easily identified by the presence of the Philadelphia chromosome (or its molecular equivalent); the myelodysplastic syndromes (MDS), which are characterized by trilineage dysplasia; chronic myeloproliferative diseases (CMPD), which include essential thrombocythemia, polycythemia vera, and agnogenic myeloid metaplasia (AMM); and atypical CMD, which includes chronic neutrophilic leukemia, chronic eosinophilic leukemia, mast cell disease, and myeloid processes that display overlapping features of MDS and CMPD (hybrid CMD). In CMPD, a diagnosis of polycythemia vera requires evidence of an erythropoietin-independent increase in red blood cell mass; the diagnosis of both AMM and essential thrombocythemia requires the exclusion of reactive causes of bone marrow fibrosis and thrombocytosis, respectively. In addition, the Philadelphia chromosome, increased red blood cell mass, and dyserythropoiesis should also be absent. Semin Hematol 38(suppl 2):1-4.

Bone Marrow Diseases↗