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The eosinophilic fibrohistiocytic lesion of the bone marrow. A mastocellular lesion in bone disease.

Seven patients are described, six with severe osteoporosis and the seventh with osteogenesis imperfecta with moderate osteoporosis. The iliac bone marrow trephine biopsy specimens of all seven showed peculiar infiltrates consisting of elongated mast cells, eosinophils, plasma cells, and varying numbers of lymphocytes. Only one patient exhibited signs of allergy with urticaria pigmentosa; the other six patients had no abnormalities that could be related to a known mast cell disease. The lesions described here are the same as those described in five patients by Rywlin as "eosinophilic fibrohistiocytic lesion in the bone marrow". However, in our methacrylate sections the fibrohistiocytes are shown to be mast cells. Although a relationship with drug hypersensitivity is disputed, the presence of the mast cells and eosinophils suggests an allergic condition.

Adult↗

CT-guided percutaneous biopsy of the cervical spine: a series of 12 cases.

Twelve patients underwent biopsy of cervical vertebral bodies under CT guidance. An accurate diagnosis was obtained in 11. No complications were observed, except for a transitory recurrent laryngeal nerve palsy. Technical problems of the cervical spine biopsy are discussed and the utility of bone biopsy with a coaxial trephine system is emphasized.

Adult↗

[Myelinopathy due to carbon monoxyde poisoning. A study in ultrastructural neuropathology (author's transl)].

A 24-year-old woman was found comatose after 2 days of cephalalgia and vomiting. An immediate diagnosis of carbon monoxyde poisoning was disclaimed when blood carbon monoxyde was found to be 1.75 ml/100. A diagnosis of acute intracranial hypertension led to trephination with ventricular punction and brain biopsy on the third day. The patient died on the eleventh day. Ultrastructural study of biopsy tissue showed nearly normal cortex, and injured white matter, with disrupted or destroyed myelin and pycnotic oligodendroglia in contrast with nearly normal axons, astrocytes, and capillaries. Autopsy showed a typical semioval center myelinopathy. After discussion of the histotoxic, vascular, and edema theories for myelinopathy pathogenesis, primary oligodendrogial lesion is considered, and correlated with the diphasic evolution often observed in the course of carbon monoxyde myelinopathy.

Adult↗

Histopathology of the bone marrow in toxic myelopathy. A study of drug induced lesions in 57 patients.

Following the introduction of numerous highly effective drugs in recent decades, haematologists are confronted with a panmyelopathy or "toxic myelopathy" originating from the exhibition of certain therapeutic regimens. Among 16,711 trephines referred to us in the last 5 1/2 years, 57 cases or 0.34 percent were found to have clear evidence of lesions caused by the ingestion of potentially toxic agents. The evaluation of the histopathology shows two groups of alterations which concern the haematopoietic parenchyma as well as the mesenchyme of the bone marrow. Different degrees of cellularity ranging from aplasia to regenerative hyperplasia and a pronounced mesenchymal reaction with proteinaceous oedema, perivascular plasmacytosis and frequent necrobiosis of neutrophilic granulocytes or cellular debris are the most conspicuous features. However, the histopathology of the bone marrow described gives no indication of the specific drug responsible and no specific suggestion of any group of drugs. Generally the histopathology allows the recognition of lesions which are induced by the toxicity of these agents. Therefore a bone marrow biopsy should be included in the diagnostic procedures whenever a toxic lesion is suspected of causing haematological disorders, particularly in all cases of uncertain pancytopenia.

Adolescent↗

Histomorphometry of bone marrow biopsies in chronic myeloproliferative disorders with associated thrombocytosis--features of significance for the diagnosis of primary (essential) thrombocythaemia.

A histomorphometric analysis was performed on trephine biopsies of the bone marrow in 55 patients with chronic myeloproliferative disorders (CMPDs) and marked thrombocytosis (platelet count exceeding 600 x 10(9)/l). This study aimed at discriminating primary (essential) thrombocythaemia (PTH) from the various other subtypes of CMPDs presenting with thrombocytosis. Following the diagnostic requirements postulated by the Polycythemia-vera-Study-Group for PTH and polycythaemia vera rubra (P.vera) and the generally accepted criteria for the establishment of chronic myeloid leukaemia (CML) and agnogenic myeloid metaplasia (AMM), our cohort of 55 patients was divided into the following subgroups: CML (16 cases), P.vera (11 cases), AMM (13 cases) and finally PTH (15 cases). Histomorphometric measurements revealed that PTH was distinguishable from the other subtypes of CMPDs with respect to several histological variables: patients with PTH had a normal amount of neutrophilic granulo- and erythrocytopoiesis as well as a non-increased content of reticulin (argyrophilic) fibers in contrast to the findings in CML, P.vera and of course AMM. Moreover, sizes of megakaryocytes and their nuclei were significantly greater in PTH and internalization of haematopoietic cells (emperipolesis) was more frequently encountered in comparison with the other subtypes of CMPDs. Deviation of the circular perimeter of megakaryocyte shape was most prominently expressed in CML and AMM, and consequently generated an increased number of a-nuclear cytoplasmic fragments. In contrast to this feature aberration of the nuclei from a circular outline occurred in a less pronounced way in CML, but was excessive in P.vera, AMM and PTH. Our morphometric evaluation demonstrates that certain histological features may serve as a valuable aid in discriminating PTH from the other occasionally thrombocythaemic subtypes of CMPDs.

Aged↗

Histomorphometry of bone marrow biopsies in primary osteomyelofibrosis/-sclerosis (agnogenic myeloid metaplasia)--correlations between clinical and morphological features.

Histomorphometry was performed on representative trephine biopsies of the bone marrow on admission of 50 patients (21 male, 29 female - age 67 years) with so-called primary osteomyelofibrosis/-sclerosis (OMF) not preceded by any other subtype of chronic myeloproliferative disorders. This study was firstly aimed at testing correlations between histological features (amount of haematopoiesis, cytological aspects of megakaryocytes, density of reticulin and collagen fibres and degree of osteosclerosis) and laboratory data, as well as spleen size and duration of relevant prediagnostic symptoms. Secondly, we concentrated on a discrimination of OMF patients into two subgroups according to bone marrow morphology and clinical variables. Statistical evaluation of histomorphometric variables and haematological findings disclosed that there was a progressive fibro-osteosclerotic process in the evolution of disease features. Increase in medullary fibrosis was significantly paralleled by an abnormal or pleomorphic megakaryopoiesis in the bone marrow: there was an increase in irregularity of perimeters for megakaryocytes and naked nuclei combined with smaller sizes of these elements including the nuclei. Additionally, there was a greater number of pycnotic bare nuclei. A number of morphometric features (density of fibres, degree of osteosclerosis, amount of haematopoiesis) were associated with corresponding clinical data (spleen size, length of preclinical history). By consideration of a set of basic histomorphometric variables our cohort of 50 patients could be divided into an early hyperplastic subtype with no or minimal medullary reticulin and another group with conspicuous fibrotic and osteosclerotic alterations of the bone marrow. It was noticeable that we found no significant correlation between amount of haematopoiesis or marrow cellularity with splenomegaly. This result suggests that splenic haematopoiesis (myeloid metaplasia) may represent an autonomous or neoplastic process and not only compensation for a failing fibro-osteosclerotic bone marrow.

Aged↗

Differentiation of plasma cell infiltrates in the bone marrow. A clinicopathological study on 80 patients including immunohistochemistry and morphometry.

In 80 patients immunohistochemical, morphometrical and clinical studies were performed on routinely referred trephine biopsies of the bone marrow showing an abnormal increase in plasma cells. From the approximately determined density of plasma cell infiltrates two main groups were distinguished, the first with an involvement exceeding 20% and the second with less than 10% of the total marrow area involved. The first group (n = 30; 324 +/- 130 plasma cells per square millimeter bone marrow) consisted of patients with frank malignant myeloma (MM) by clinical and histomorphological diagnosis. The second group (n = 50; 132 +/- 54 plasma cells per square millimeter bone marrow) with plasmacytic differentiation of infiltrates, had to be further divided into one component with evidence for initial or residual MM following chemotherapy (n = 27), another with obviously monoclonal gammopathy of undetermined significance--benign monoclonal gammopathy (BMG, n = 6), and a final set of cases with a reactive plasmacytosis mostly associated with an inflammatory condition (n = 17). There was an excellent agreement between the intracellular immunoglobulin staining as defined by the immunoperoxidase technique and the serum or urinary M-component detected by immunoelectrophoresis. In MM significant correlations were found between osteoclastic activity (number of osteoclasts specifically stained by acid phosphatase) per trabecular bone area, presence of lytic bone defects and the density of plasma cell infiltrates in the marrow. This latter feature corresponded well with the titer of secreted serum M-components measured by quantitative immunoelectrophoresis. Using morphological data alone, BMG cases could not be discriminated with any certainty from initial or residual plasmacytic MM. They consequently need a prolonged clinical follow up to clarify the nature of the lesions.

Bone Marrow↗

Short-term lack of cerebral perfusion with good outcome. Advantages of early intracranial pressure monitoring.

A 21-year-old man was injured by a tailboard of a truck. He suffered a severe head injury with bilateral depressed skull fractures necessitating surgical decompression. On admission to the hospital the patient showed bending to pain stimuli (Glasgow Coma Score 5). Anisocoria was noticed from the beginning. Initial intracranial pressure (ICP), measured 3 hours after injury, was 30 mm Hg, and the cerebral perfusion pressure (CPP) was 70 mm Hg. During surgical elevation of the skull fracture on the right side an un-explainable rise of ICP to values of 100 mm Hg occurred, which corresponded to the mean arterial blood pressure (MAP). At the same time both pupils were dilated and fixed indicating a lack of cerebral perfusion. Due to immediate trephination of the opposite side, the ICP was lowered to values below 20 mm Hg, and sufficient cerebral perfusion (above 50 mm Hg) was regained. The patient showed a good recovery and was transferred to a rehabilitation center 5 weeks after injury. This case report emphasizes the importance of early and continuous intracranial pressure monitoring for adequate therapy in neurosurgical emergencies.

Adult↗

[Urgent acute situations for the general surgery department: craniocerebral trauma].

In the management of accident patients a physician must be able to recognize the life-threatening complications of craniocerebral trauma. Therefore, he must be well versed in the symptomatology of such complications and by a short neurological examination be able to find out the risk to the patient. Only in this way is it possible to decide whether the patient has to be merely observed or additional neuroradiological investigation is necessary, whether he has to be transferred to a neurosurgical department or in emergency cases be operated on immediately. In acute epidural hematoma, e.g., with signs of midbrain compression, an instantaneous trephination is imperative; therefore, a general surgeon, too, should have the ability to perform such an operation, the technique of which is described in the paper.

Brain Injuries↗

The state of the frontal sinus after craniotomy.

In 39 patients, 24 male, 15 female, the frontal sinus was opened during a frontal, a bifrontal or a pterional trephination for neurosurgical treatment of trauma, tumours or aneurysms. In 25 out of 39 patients the frontal sinus subsequently was obliterated, in 11 out of 39 patients the apical half or 2/3 of the sinus were ablated and the remaining caudal part covered with a galea periosteal flap leaving the fronto-nasal duct intact. In 3 out of 39 patients, the periphery of the sinus was ablated and the remaining part covered. All patients were followed over a mean period of 2 years and had a final evaluation using CTs, X-rays, ultrasonography and a complete ENT examination. In 4 out of 25 obliteration was successful and complete, in 16 out of 25 there was a partial or complete re-aeration of the residual sinus and in 5 out of 25 the sinus was infected. In one out of eleven patients where the frontal sinus was bisected with preservation of the lower half, the basal part of the sinus was infected, in 10 out of 11 the residual sinus was aerated and draining well. In one out of three patients where the periphery of the sinus was ablated, there was infection, two of three sinuses were aerated. Evaluating the experience in these 39 patients and our recent experience, obliteration of the sinus with muscle is very unreliable and the use of bone wax is obsolete. Covering a bisected or peripherally opened sinus with fascia or a galea periosteal flap will yield a well aerated and draining sinus, provided the naso-frontal duct is intact.

Adolescent↗

Ventricle puncture for external CSF drainage and pressure measurement using a modified puncture needle.

A modified needle for external ventricular drainage is presented. Contrary to conventional spinal needles with this instrument the sharp guide can be withdrawn after penetration of the dura, thus, no sharp instrument affects the brain, nor remains within the ventricles in cases of continuous drainage. Furthermore, depth of penetration can be determined preoperatively by means of a set screw also facilitating fixation of the needle. To date, we have used this modified needle in 50 patients for short or long term CSF drainage and for CSF pressure measurements. Trephination was performed with a hand or battery-driven drill. Except for an infection in one case we found no serious complications in our patients. In our opinion, ventricle puncture for external drainage and pressure measurement with this device is a simple and safe method and can be performed on the ward under local anaesthesia.

Adolescent↗

Chronic subdural haematoma--a comparison of two different treatment modalities.

Burr-hole craniotomy (BHC) and closed-system drainage undoubtedly is currently the most accepted treatment offered in chronic subdural haematoma (CSDH). Although twist-drill trephination (TDT) techniques have been available for years, now a special subdural catheter kit has been launched for treatment of CSDH. In a prospective study, 33 patients with 36 CSDH were treated with a 5-mm TDT regimen and insertion of a CORDIS subdural catheter (CORDIS Corp., Miami, USA). The results are compared with a consecutive series of 33 patients treated previously with an 11-mm BHC and closed-system drainage for 40 CSDH: Recurrence and persistence rate of CSDH treated with TDT necessitating a second intervention was 18.1%, no further surgical intervention was necessary. In BHC treated patients, 33.3% of haematomas had to be reoperated on, another 6.0% had to be re-operated on a third time. Infection rate in BHC treated patients was 18.1% as compared with a 0% infection rate in patients treated with the TDT technique. Mortality rate for the BHC method was 9.0% as compared with 6.0% in the TDT treatment regimen. Significantly better clinical results are achieved using the TDT technique with insertion of a special subdural catheter, making this procedure superior to the BHC regimen.

Adult↗

Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias.

We studied 21 bone marrow specimens from 21 patients with systemic lupus erythematosus (SLE) and peripheral cytopenias: anaemia (Hb < 10 g/dl), and/or leucopenia (white blood cell count < 4 x 10(9)/l), and/or thrombocytopenia (platelets < 150 x 10(9)/l). None of the patients had used immunosuppressive drugs in the 2 months before the study, and 11 (52.4%) had never used these drugs. The global and specific series cellularity, degree of fibrosis and necrosis were evaluated by bone marrow trephine; morphological abnormalities and iron stores were evaluated by cytological smears. The most important abnormalities viewed in bone marrow biopsies were: global hypocellularity (47.6%), increased reticulin proliferation (76.2%) with myelofibrosis in one patient, and necrosis (19.0%). The marrow aspirates were difficult to obtain in four patients, who showed an increased reticulin proliferation on histological analysis. Plasmocytosis was present in 26.7% of cases and in one there was a serum monoclonal component (IgG kappa). Iron stores were normal or increased in 26.7% of specimens and decreased or absent in 73.3%. The most frequent peripheral abnormality was leucopenia in 90.4% (19/21) and granulocytic hypoplasia was observed in 47.3% (9/19) of these patients. We conclude that the bone marrow may be a target organ in SLE with cytopenias.

Adolescent↗

Myelodysplastic syndromes: immunohistochemical and morphometric evaluation of proliferative activity in erythropoiesis and endoreduplicative capacity of megakaryocytes.

An immunohistochemical and morphometric analysis was performed on bone marrow trephine biopsies in 40 patients with primary myelodysplastic syndromes (MDS) to evaluate the proliferative activity in erythropoiesis and the endoreduplicative capacity of megakaryocytes. Control groups included normal bone marrow and marrow from cases presenting with pernicious anaemia. Double-immunostaining was applied with a monoclonal antibody (PC10) directed against proliferating cell nuclear antigen (PCNA), followed by antibodies against glycophorin C (Ret40f) or platelet glycoprotein IIIa (Y2/51-CD61) for the identification of the erythroid and megakaryocytic cell lineage. Comparison with normal bone marrow showed a reduction of erythropoiesis accompanied by an increase in atypical (micro-) megakaryocytes. Erythroid precursors displayed significant enhancement of PCNA-immunostaining. Megakaryocytes showed no increase in the relative frequency of PC10-positive cells (PCNA-labelling index). In pernicious anaemia, predominance of macrocytic-megaloblastoid erythropoiesis was associated with a striking increase in PCNA-labelling. Cell kinetic studies in this disorder revealed an abnormal arrest, particularly in S-phase which generates an over-expression of PCNA. Similar conditions were believed to be present in MDS with secondary folate deficiency. This mechanism explains the relatively high rate of positively-reacting pro- and erythroblasts which is not invariably accompanied by an increase in cell proliferation. Determination of megakaryocyte size and PCNA-staining capacity resulted in a significant increase in PC10-positive cells among micromegakaryocytes. Our findings on this cell lineage are in keeping with the assumption of a block in endoreduplicative activity at higher ploidy levels, associated with an apparently not-deregulated endomitosis in small-sized megakaryocytes of lower ploidy stages.

Aged↗

Temporal lobe lesions in head injury.

In cases of closed head injury temporal lobe lesions e.g. contusion, laceration, pulping, or intracerebral haematoma frequently result in an expanding process. These are frequently associated with an overlying subdural haematoma. Eighty five cases of such lesions from a consecutive series of 1,000 cases of head injury have been analyzed. The lesions are caused by severe injury resulting in loss of consciousness and skull fracture. Most of them are contre-coup lesions. Clinically they manifest themselves like any other acute or subacute intracranial hematoma. The majority of the patients have contralateral hemiparesis and pupillary abnormalities. Carotid angiography is valuable for diagnosis. These lesions are likely to be missed when exploratory burr holes are made or, are erroneously diagnosed as acute subdural haematoma or brain oedema. Smaller lesions, not showing progressive deterioration, may respond to conservative treatment. However, surgical decompression is essential in most cases A frontotemporal osteoplastic craniotomy or a large Scoville trephine hole is essential to deal with these lesions adequately.

Accidents, Traffic↗

Central corneolimbal transplantation under systemic ciclosporin A cover for severe limbal stem cell insufficiency.

BACKGROUND: Severe stem cell deficiencies uniformly lead to superficial conjunctivalization of corneal grafts with subsequent functional failure. We sought better long-term results by transplanting central corneolimbal grafts and simultaneously protecting the graft and its stem cells from immunological destruction by means of systemic administration of ciclosporin A. PATIENTS AND METHODS: In an ongoing pilot study, up to April 1995 20 eyes with stem cell dysfunctions of various etiology (e.g. chemical burn, ocular pseudopemphigoid, congenital aniridia) received eccentrically trephined fresh corneal grafts of 7.7-10.0 mm diameter. About one third of the circumference of the grafts contained limbal area. The mean age of the patients was 46.2 years (range 9-84 years). All patients received systemic ciclosporin A for at least 12 months. At present, the mean follow-up period is 9.6 months (mean 1-20.6 months). RESULTS: Fourteen of 20 grafts (70%) have remained clear so far. Reasons for six graft failures were surface disorders in four eyes, immune reactions in one eye and surface disorders in combination with immune reactions in another eye. Ten of 20 grafts (50%) experienced severe surface disorders. In six eyes surface disorders were coincident with endothelial immune reactions, in four eyes they were not. In four of 20 grafts (20%) conjunctivalization was observed in front of the transplanted limbal area; in seven of 20 grafts (35%) conjunctivalization occurred only distant from the transplanted limbal stem cells. CONCLUSIONS: Our method of central corneolimbal transplantation with simultaneous protection of the transplanted stem cell population from immunological destruction by means of systemic ciclosporin A has been successful for 14 eyes with severe stem cell deficiencies up to 20.6 months postoperatively. This new treatment principle promises - for the first time - long-term rehabilitation for a majority of eyes with severe limbal stem cell deficiencies.

Adolescent↗

[The electro-oculogram in the early postoperative period following fistulizing operations (author's transl)].

EOGs were obtained from 2 groups of patients suffering from glaucoma chronicum simplex the day before surgery and 3, 5, 8, 11 and 14 days after trephining or fistulizing operation after Scheie. Though and peak potentials of the treated eyes were significantly less than those of the untreated fellow eyes five days after surgery. In addition on the 11. postoperative day after Scheie a second potential decrease was seen. The EOG-changes observed appear to reflect disturbances of the choroidal circulation after fistulizing operations.

Aged↗

Trabecular bone structural variation in biopsy sites of the beagle ilium.

Iliac trabecular bone sites were studied in 2 male and 2 female adult beagles to determine normal structural variability among different sites, and to identify transilial biopsy sites with minimum structural variation. Only sites that provided adequate amounts of trabecular bone were studied. Trabecular bone area and perimeter were measured on microradiographs of ground sagittal sections using a Quantimet 720 image analyzer, and percent bone, perimeter to area ratio, and mean trabecular width were calculated. Over 100% variation was found in the parameters studies among sections from different sites within the same animal. However, variability was minimal among sections obtained more than 2 mm from the lateral or medial cortex within the most caudal sites evaluated. Two sites appropriate for sampling with an 8 mm trephine were identified as being centered over the following points: (a) a point 7 mm caudal to the cranial dorsal spine and 7 mm ventral to the tuber sacrale, and (b) a point 7 mm caudal to the cranial ventral spine and 7 mm dorsal to the tuber coxae. Comparison of data obtained from males and females indicated that sampling variation may also be reduced by using dogs of one sex and by preferential use of males.

Animals↗