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Synonym: Zinsser-Cole-Engman syndrome the onset of dyskeratosis congenita is between early childhood and puberty antibiotic yellow teeth cheap generic cefadroxil uk. The main symptoms are cutaneous reticulated pigmentation virus y antivirus purchase discount cefadroxil, atrophy and thinning of the nail plate bacterial cell diagram cheap cefadroxil 250mg on-line, and oral leucoplakia antibiotic xy purchase genuine cefadroxil on-line. Deformity of the nail plate occurs first, followed by reticular pigmentation on the neck region spreading to the 354 20 Nevus and Neurocutaneous Syndrome trunk and extremities. Leukoderma keratosis-like change appears most frequently on the tongue, buccal mucous membranes and genitalia, and it tends to become malignant. It is accompanied by progressive aplastic anemia, splenomegaly and esophageal blockage. The main treatments are excision for leukoplakia and sympClinical images are available in hardcopy only. Epidermal nevus syndrome Epidermal nevus syndrome is unilateral epidermal nevus accompanied by central nervous abnormalities such as mental. Cutis marmorata telangiectasia congenita Livedo reticularis appears at the time of birth or shortly thereafter. Deformity occurs in the central nerves, heart, blood vessels, muscles, skeleton and eyes in nearly half of cases. Reticularis disappears with age, and most cases resolve within 2 years after birth. Hemangioof intermediate malignancy, and malignant vascular tuendothelioma is a neoplasm of endothelial cells that can mors. These are found predominantly in younger tumor with high local recurrence rate and risk of dischildren and adolescents. Classification sparse, probably due to the tendency of these lesions to of these lesions is still the source of much controversy involve skin and superficial tissue, in contrast to other and is based on clinical appearance, pathology, embrysoft tissue sarcomas. The majority of hemangiomas do not need treatIn the past it was debated whether vascular tumors are ment. In the On the other hand, many vascular lesions show no nineteenth century vascular lesions were thought to be cellular proliferation; they usually are not present at ’produced by the longing of the mother, for particular birth but grow with the child and have no involution things,or her aversion to them’. These lesions are called vascular malformations, vus materneus or stigma metrocelis were in reference to and they are divided into capillary, venous, arterial and the mother. Because vascular tumors sometimes closely lymphatic types depending on the predominant vessel resemble normal vessels, it is difficult to distinguish type [42]. One exview of this limitation no attempt is made to separate hibits a rapid growth phase followed by a period of stamalformations from benign neoplasms. All lesions are bilization and finally involution; these show a female called hemangiomas, and hemangioma is defined as ’a benign but nonreactive process in which there is an inTable 16. Differentiating features of hemangiomas and vascular crease in the number of normal or abnormal-appearing malformations (modified from [65]) vessels [12]. Hemangiomas may be either of two types: Hemangiomas Vascular malformations those localized in one area and those involving large segments of the body. The histological classification of Exhibit cellular proliferation Comprised vascular tumors proposed by Enzinger et al. Small or absent at birth Present at birth Localized hemangiomas are the more common. They Rapid growth during infancy Growth proportional have been classified according to clinical, embryologic, to child or pathological criteria but no system is entirely satisInvolution during childhood No regression factory. Therefore, they classify lymphangioma as a vascuthe predominant vessel type, the former are grouped as lar tumor. Intramuscular hemangioma usually shows an overgrowth of adipose tissue, giving the impression of the term hemangioendothelioma is currently used for angiolipoma. Unlike their cutaneous variants, it is not lesions which are histologically intermediate in appearalways possible to make a clear distinction between difance between hemangiomas and angiosarcomas. A classi According to Enzinger,the three types are epithelioid cation into small-vessel, large-vessel and mixed types hemangioendothelioma, spindle cell hemangioendowith different clinical behavior was proposed by Allen thelioma and malignant endovascular papillary hemto avoid misinterpretation since some of the small-vesangioendothelioma. Only the epithelioid subgroup ocsel type lesions show alarming histological features recurs in deep soft tissues,most commonly of the extremsembling those of a sarcoma [1]. The other types develop preferentially in the Angiomatosis is a rare condition in which large segdermis or subcutaneous tissues [13]. A characteristic feasive tumors of intermediate malignancy and rarely ture of this condition is the large amount of mature fat metastasizing types. It most commonly and angiomatosis is made better by clinical than pathooccurs in the retroperitoneum or the skin. In this classification, no disanaplasia makes it difficult to distinguish from carcinotinction is made between benign vascular neoplasms or mas. Similarly, it is impossible to vascular channels lined by atypical endothelial cells. Ramon the terms hemangiosarcoma and lymphangiosarcoma ic problems or threaten vital structures. Kaposi’s sarcomas have spindle cell areas containsenile angioma are clinical variants of the same subtype ing vascular channels. It presents as cutaneous leage and anatomical distribution with the capillary sions in the form of multiple patches. The malignancy and epithelioid hemangioendothelioma is majority require surgical resection. They are distinguished from the capillary and cavernous counterparts by the presence of thick-walled 16. On the other hand, the glomus tumor is a neoplasm consisting of cells shunting of blood causing cardiac overload, pain and which closely resemble smooth muscle cells of the norhypertrophy of the involved extremity are problems mal glomus body. The glomus body is an arteriovenous associated with deep lesions and require an adequate anastomosis that has an important role in thermoregutherapeutic approach. It is located in the subungual region, digits, and Epithelioid hemangioma and granulation type hempalms. The lesion shows both muscle fibers and epitheangioma are unusual vascular tumors. Epithelioid hemangioma consists of well formed but immature vessels and a prominent infiammatory component. Hemangiopericytoma is an uncommon tumor, first deCompared to superficial hemangiomas, deep-seated scribed by Stout and Murray,and is composed mainly of lesions are quite uncommon, with a reported frequency pericytes [8,15,26,28,33,44,52,55,63]. Pain is reported to be more common in tumors involving long, narrow musTo avoid confusion we follow here a combination of the cles, probably through stretching of the muscle fibers. Lesions have often been present for many years and it the differentiation of Mulliken and Glowacki beis therefore likely that many examples are congenital. Therapy is aimed at complete excision, make up about 7% of all benign soft tissue tumors. IntramusMost of the lesions, however, are located in the skin or cular hemangiomas are benign, but with a high incisubcutaneous tissues, while only a minority is deeply dence of local recurrence. Synovial hemangioma and hemangioma of peripherCapillary hemangiomas form the largest group. The synovial type almost always incept for cherry angioma they occur predominantly in volves the knee joint, presenting with pain, swelling childhood. The most common site is the supraof capillary hemangioma with a characteristic clinical patellar pouch. They occur mostly in superficial tissue of the joint, a radiographic appearance identical to that of head and neck region and arise a few weeks after birth. Only a small They grow slowly to reach a maximal size at six months proportion of lesions are correctly diagnosed before of age and then regress. Chapter 16 Tumors and Tumor-like Lesions of Blood Vessels 267 Kasabach-Merritt syndrome was first reported in 1954 [25]. The cardinal features of this syndrome include an enlarging hemangioma, thrombocytopenia and microangiopathic hemolytic anemia with acute or chronic consumptive coagulopathy. Many therapeutic modalities have been applied, aiming at a twofold objective: to control the bleeding and to reduce the size of the lesion [11, 25, 32, 38]. Maffucci’s syndrome is a rare dysplasia characterized by multiple hemangiomas and enchondromas. The bones are shortened and have multiple exostoses and enchondromas [6, 12].

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The definitive host becomes infected upon eating freshwater crabs or crayfish that contain metacercariae antibiotics history buy generic cefadroxil 250mg online. Once in the intestine antibiotic quadrant discount cefadroxil uk, the metacercariae are released from their envelope and penetrate the intestinal wall antibiotic names for uti order cefadroxil 250mg on line, remain in the peritoneal cavity for several days antibiotic resistant germs buy cheap cefadroxil 250 mg, and then migrate through the diaphragm into the pleural cavity. Although Paragonimus trematodes are morphologically hermaphroditic, functionally they are unisexual and, with the exception of the triploid forms of P. Juveniles that do not find a mate usually continue to move around in the pleural cavity or the lungs and cause further damage, while the adults in the pulmonary cysts are usually found in pairs. When metacercariae are ingested by an inappropriate host—for example, a wild boar, rabbit, or rodent—the parasites remain inside without developing further and utilize the animal as a transfer, or paratenic, host. The wild boar (Sus scrofa leucomystax) appears to serve as a paratenic host for P. Geographic Distribution and Occurrence: Paragonimus is found throughout the world. The geographic distribution of species that affect humans is indicated above in the section on etiology. A study conducted in several provinces of Thailand revealed an infection rate of 6. A sizable endemic area was identified in Vietnam, in which 44 of 155 patients (28%) with chronic pulmonary disease were found to be infected with Paragonimus (Queuche et al. In a survey conducted in an endemic region of Cameroon, examination of sputum or feces revealed P. Human cases of the disease have been seen in Colombia, Costa Rica, Ecuador, El Salvador, Honduras, Mexico, and Peru (in Cajamarca and along the coast north of Lima). In Ecuador, between 1921 and 1969, a total of 511 cases were reported, and between 1972 and 1976, there were 316 cases in four provinces of that country, most of them in the province of Manabi (Arzube and Voelker, 1978). In a study carried out in northwestern Ecuador, 43% of the crayfish examined were found to be infected, and 62% of the streams proved to be harboring infected crustaceans (Vieira, 1992). About 20 cases have been diagnosed in Cajamarca, Peru, and some have also been reported in Mexico. A long time elapses between the ingestion of metacercariae and the appearance of symptoms, though the duration of this period is variable. The parasites can cause damage as they migrate toward the lungs and seek a mate in the pleural cavity, while they are encysted in the lungs, and sometimes when they become lodged in ectopic sites. Indeed, experimental studies in dogs have shown that migration toward the lungs can produce considerable damage. The prominent symptoms of pulmonary paragonimiasis are chronic productive cough, thoracic pain, blood-tinged viscous sputum, and sometimes fever (Im et al. Small numbers of parasites in the lungs do not significantly affect the health of the patient and do not interfere with routine activity. About two-thirds of the shadows revealed by radiography are located in the middle and lower portions of the lungs; they are rarely seen in the apex. According to reports of cases in the Americas, the brain has also been parasitized by species other than P. In the Republic of Korea, which is a hyperendemic area, an estimated 5,000 cases of cerebral paragonimiasis occur each year. The symptomatology is similar to that of cerebral cysticercosis, with cephalalgia, convulsions, jacksonian epilepsy, hemiplegia, paresis, and visual disorders. Abdominal paragonimiasis produces a dull pain in that region, which may be accompanied by mucosanguineous diarrhea when the intestinal mucosa is ulcerated. In other localizations, the symptomatology varies depending on the organ affected. The subcutaneous nodular form, characterized by intense eosinophilia, is predominant in infections caused by P. In addition to migratory subcutaneous nodules, the most common manifestations of P. Cases of ectopic paragonimiasis in the brain, liver, and perivesical and cutaneous fat have been observed in Latin America. Twelve cases of cutaneous paragonimiasis occurred in the same family in Ecuador; in addition, there was a single isolated case in that country and another in Honduras (Brenes et al. The symptoms are similar to those of human pulmonary paragonimiasis, with coughing and bloody sputum. In the laboratory, trematodes appear in the lungs of dogs 23 to 35 days after experimental infection. The parasitosis begins as pneumonitis and catarrhal bronchitis, which are followed by interstitial pneumonia and the formation of cysts. Transmission results from the ingestion of raw or undercooked crustaceans, raw crabs marinated in wine (“drunken crabs”), or crustacean juices. Paragonimiasis is a public health problem in countries where it is customary to eat raw crustaceans or use them for supposedly therapeutic purposes. However, the disease is a problem in Japan as well, even though crustaceans are well cooked before they are eaten; in this case, the main source of infection is hands and cooking utensils contaminated during the preparation of crustaceans. It is possible that man may also become infected by eating meat from animals that are paratenic hosts carrying immature parasites, as evidenced by cases on the island of Kyushu, Japan, that occurred following the consumption of raw wild boar meat. The hypothesis that there are paratenic hosts is reinforced by the fact that paragonims have been observed in carnivores such as tigers and leopards that do not eat crustaceans (Malek, 1980). Transmission is always cyclic—the infection cannot be transmitted directly from one definitive host to another. The parasite must complete its natural cycle, and in order for this to happen the two intermediate hosts must be present—appropriate species of both snails and crustaceans. In endemic areas of eastern Asia, the human infection rate is high enough that man can maintain the infection cycle alone through ongoing contamination of freshwater bodies with human feces. In such areas, the role of animal definitive hosts may be of secondary importance. This experience bears out the importance of human infection in maintaining the endemic. On the other hand, in several parts of Africa, Latin America, and Asia, wild animals are more important than man or domestic animals in maintaining the infection cycle. Diagnosis: In endemic areas, paragonimiasis may be suspected if the typical symptoms are present and the consumption of raw or undercooked crustaceans is a local custom. Radiographic examination is useful, but the findings may be negative even in symptomatic patients. Moreover, interpretation of the results can be difficult in nonendemic areas because the images may be mistaken for those of tuberculosis. Specific diagnosis of pulmonary paragonimiasis is based on the identification of eggs in sputum, fecal matter, pleural effusions, or biopsies. The eggs are reddish brown, operculate, and enlarged at the end opposite the operculum. It is important to differentiate the eggs of Paragonimus from those of other trematodes, as well as cestodes of the order Pseudophyllidea, such as Diphyllobothrium. The cerebral forms can be mistaken for tumors or cysticercosis, and the cutaneous forms, for other migratory larvae—hence the interest in developing indirect tests. An intradermal test that was only weakly sensitive and of questionable specificity was widely used in the past for epidemiologic purposes. In a province of China, a 1961 study found that 24% of the persons examined had positive skin tests, and almost half of those cases were confirmed. This assay can distinguish infections caused by different species of Paragonimus (Kong et al. In addition, the polymerase chain reaction is being used to diagnose paragonimiasis (Maleewong, 1997). Control: In endemic areas, control efforts should be directed at interrupting the infection cycle by the following means: a) education of people to prevent the consumption of raw or undercooked crabs or crayfish; b) mass treatment of the population to reduce the reservoir of infection; c) elimination of stray dogs and cats for the same purpose; d) sanitary disposal of sputum and fecal matter to prevent the contamination of rivers; and e) controlling snails with molluscicides in areas where this approach is feasible. For a control program to be effective, it should encompass the entire watershed area and adjacent regions. In Latin America, where the transmission cycle appears to occur predominantly in wildlife and where human cases are sporadic, the only practical measure is to educate and warn the population about the danger of eating raw or undercooked crustaceans. A study in China investigated the possibility of destroying metacercariae in crustaceans by irradiation with cobalt-60. No parasites could be recovered from mice infected with metacercariae irradiated at 2.

These disposed in a central antimicrobial vinyl chairs 250 mg cefadroxil overnight delivery, slender virus your current security settings cheap cefadroxil online amex, wavy ribbon (hence the designation “Caterpillar cells” antibiotic resistance new zealand cheap cefadroxil 250 mg with visa. Some of the larger macrophages becomes multinucleated to virus warning cefadroxil 250mg generic form aschoff giant cells. All of the following statements are true regarding reversible cell injury, except: 1. Tartarate resistant acid phosphatase positivity is typically seen in hairy cell leukemia. Robbins /50 Vascular changes in acute inflammation 1) Vasodilation is one of earliest manifestation. The subtype of Hodgkin’s disease, which is histogenetically distinct from all the other subtypes, is: 1. In familial Mediterranean fever, the gene encoding the following protein undergoes mutation: 1. Patients with IgD myeloma may present with no evident M-spike on serum electrophoresis. A diagnosis of plasma cell leukemia can be made if circulating peripheral blood plasmablasts comprise 14% of peripheral blood white cells in a patient with white blood cell count of 11 x 109/L and platelet count of 88 x 109/L. In a patient with multiple myeloma, a monoclonal light chain may be detected in both serum and urine. But iwhen the ward leukemia is used, it always means that cells are > 20% of circulating W. Which one of the following serum levels would help in distinguishing an acute liver disease from chronic liver diseasefi Serum albumin has a long half-life: 15 to 20 days, with approximately 4% degraded per day. Because of this slow turnover, the serum albumin is not a good indicator of acute or mild hepatic dysfunction; only minimal changes in the serum albumin are seen in acute liver conditions such as viral hepatitis, drug-related hepatoxicity, and obstructive jaundice. Hypoalbuminemia is more common in chronic liver disorders such as cirrhosis and usually reflects severe liver damage and decreased albumin synthesis. One exception is the patient with ascites in whom synthesis may be normal or even increased, but levels are low because of the increased volume of distribution Q. Which one of the following diseases characteristically causes fatty change in liverfi On examination, cervical lymph nodes were found enlarged and spleen was palpable 2 cm below ithe costal margin. Katzung /472,473 Drug induced parkinsonism is treated, when necessary, with conventional antiparkinsonism drugs of the antimuscarinic type or, in rare case with amantadine. First step is tried to discontinue or reduce the current antipsychotic or switch to one of the newer atypical agent. Finasteride blocks the conversion of testosterone to dihydrotestosterone, the principal androgen in the prostate. Combined treatment with terazosin has proved no better than treatment with an alpha blocker alone in most men. But also used in scleroderma, Wegener’s granulomatosis, giant cell arteritis, sarcoidosis. In unconjugated hyperbilirubinemia, the risk of kernicterus increases with the use of: 1. Sulphonamide Number of allergic reactions hematologic complications Maculopapular rashes urticaria Granulocytosis, Life-threatening reactions such as erythema Hemolytic and megaloblastic anemia multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis Granulocytopenia Trimethoprim Hyperkalemia Renal insufficiency caused by crystaluria Jaundice and kernicterus in newborns Drug fever with serum sickness, hepatic toxicity (including necrosis), and systemic lupus erythematosus. The severe hypersensitivity reactions occurred most commonly after treatment with the long-acting sulfonamides, such as sulfamethoxypyridazine Photosensitivity reactions are also relatively common with sulfonamides. Hemolytic anemia is most common in patients with glucose-6-phosphate dehydrogenase deficiency who take long-acting compounds; trimethoprim-sulfamethoxazole rarely causes hemolysis in such subjects. Renal insufficiency, caused by crystals of the relatively insoluble acetyl metabolite, is observed primarily with the long-acting sulfonamides. Oculogyric crisis is known to be produced by all of the following drugs except: 1. Bnrown 9th ed/384-85 Acute extrapyramidal side effects of antipsychotic are Dystonia abnormal movements of the tangue and facial muscles with Fixed postures and spasm, Include torticolis called Oculogyric crisis And bizarre eye movement Hence answer is (2) atropine Remaining three are the typical antipsychotic that causes the acute dystonia. Clozapine and olanzopine are the most likely of ithe atypical agents to cause anticholinergic (antimuscarinic) side effects. Risperidone and amisulpride are as likely as classical antipsychotic to raise prolactin level and causing galactorrhea. Rizatriptan appears to be the fastest acting and most efficacious of the triptans currently available. Sumatriptan and zolmitriptan have similar rates of efficacy as well as time to onset, whereas naratriptan is the slowest acting and the least efficacious. The advantage of tacrolimus are minimizing episodes of rejection, reducing the need for additional glucocorticoid doses, and reducing the likelihood of bacterial and cytomegalovirus infection. In most transplantation centers, tacrolimus has now supplanted cyclosporine for primary immunosuppression, and many centers rely on oral, rather than intravenous, administration from the outset. The toxicity of tacrolimus is similar to that of cyclosporine; nephrotoxicity and neurotoxicity are the most commonly encountered adverse effects, and neurotoxicity (tremor, seizures, hallucinations, psychoses, coma) is more likely and more severe in tacrolimus-treated patients. Both drugs can cause diabetes mellitus, but tacrolimus does not cause hirsutism or gingival hyperplasia. Because of overlapping toxicity between cyclosporine and tacrolimus, especially nephrotoxicity, and because tacrolimus reduces cyclosporine clearance, these two drugs should not be used together. Penicillins those are not penicillinase susceptible are methicillin, Cloxacillin, naficillin etc. Answer is 3 Artemether-lumefantrine and atovaquone-proguanil are recently introduced, well-tolerated antimalarial drugs used in 3-day regimens. Answer is 3 Synthetic opioids, sharing properties of opium and morphine, include meperidine, propoxyphene. Various opiate effects are analgesia, respiratory depression, constipation, and euphoria. Although various derivatives and congeners of atropine (such as propantheline, isopropamide, and glycopyrrolate) have been advocated in patients with peptic ulcer or with diarrheal syndromes Neostigmine is Cholinesterase inhibitors that enhance the effects of parasympathetic stimulation and leads to reversal of intoxication by agents with a anticholinergic action Q. Medial longitudinal eye with complaints of pain, fasciculus photophobia, and blurring after c. The treatment consists of corneal scrapings were cultured all of the following except over chocolate agar and blood a. In a patient presenting with readings when they are hemoptysis the x-ray was found used together to be normal. Obliteration of hospital and is argumentative on retrosternal shadow on being questioned on her doings. Surgery is required in There is a history of a family treatment member having disappeared d. He seems to be pregnancy talking to himself and sometimes muttering to himself loudly. Simvastatin is extensively abnormality in which of the metabolised and Provastatin is least following colours c. IgG antibodies against raphe between the buccinator and toxoplasma in the mother a. Middle pharyngeal of External carotid artery is constrictor arising from the medial side a. In a mutation if valine is replaced past few months with atrophy of by which of the following would the thenar eminence on not result in any change in the examination. Homoestatic mechanisms in a concentration of 2mg in the body system depends upon all of afferent arteries and the veins the following except contain 0 mgs and excreted with a. To prevent neonatal acidosis infection which of the following needs to be given to the newborn 77. Transposition of great was passing golden yellow soft vessels with intact septum stools, and was found to be b. In which of the following examination the fingers could sterilization procedures is best feel the anterior fontanelle and reversible the superior orbital ridges. Enantiomeric drugs are vascular resistance used in racemic mixtures, which have different pharmacokinetic and pharmacodynamic properties 97.


  • Congenital generalized fibromatosis
  • Kozlowski Ouvrier syndrome
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The characteristic feature of the cells antibiotics for sinus infection safe while breastfeeding cefadroxil 250 mg without a prescription, which are usually quite large with variably structured nuclei treatment uti zithromax discount cefadroxil 250mg overnight delivery, is extensive promyelocytic granulation infection jokes generic cefadroxil 250mg free shipping. Acute leukemia with predominantly bilobed nuclei is classified as a variant of M3 (M3V) antibiotics for uti types order generic cefadroxil on line. The cytoplasm may appear either ungranulated (M3) or very strongly granulated (M3V). Given the close relationship between cells in the granulopoietic and the monocytopoietic series (see p. Thus, acute myelomonocytic leukemia shows increased granulocytopoiesis (up to more than 20% myeloblasts) with altered cell morphologies, together with increased monocytopoiesis yielding more than 20% monoblasts or promonocytes. Immature myeloid cells (atypical myelocytes to myeloblasts) are found in peripheral blood in addition to monocyte-related cells. Cytochemically, the classification calls for more than 3% peroxidase-positive and more than 20% esterase-positive blasts in the bone marrow. M4 is similar to M2; the difference is that in the M4 type the monocyte series is strongly affected. In addition to the above characteristics, the M4Eo variant shows abnormal eosinophils with dark purple staining granules. The diagnosis of acute leukemia is relevant even without further subclassification a b c e d f. Auer bodies d Bone marrow cytology in acute myelomonocytic leukemia M4: in addition to myeloblasts (1) and promyelocytes (2) there are also monocytoid cells (3). Two morphologically distinct forms of acute monocytic leukemias exist, monoblastic and monocytic. Often, only the faintly grayish-blue stained cytoplasm hints at their derivation. In monocytic leukemia (type M5b), the bone marrow containspromonocytes, which are similar to the blasts in monocytic leukemia, but their nuclei are polymorphic and show ridges and lobes. The peripheral blood contains monocytoid cells in different stages of maturation which cannot be distinguished with certainty from normal monocytes. Both types are characterized by strong positive esterase reactions in over 80% of the blasts, whereas the peroxidase reactivity is usually negative, or positive in only a few cells. It is suspected when mature granulocytes are virtually absent, but blasts (myeloblasts) are present in addition to nucleated erythrocyte precursors, usually erythroblasts (for morphology, see p. The bone marrow is completely overwhelmed by myeloblasts and erythroblasts (more than 50% of cells in the process of erythropoiesis). Sporadically, some cases show granulopenia, erythroblasts, and severely dedifferentiated blasts, which correspond to immature red cell precursors (proerythroblasts and macroblasts). Acute leukemias may also derive from monoblasts or erythroblasts a b c d e f. This image of bone marrow cytology in M6 shows increased, dysplastic erythropoiesis. It can also occur as “acute myelofibrosis,” with rapid onset of tricytopenia and usually small-scale immigration into the blood of dedifferentiated medium-sized blasts without granules. Criteria for dysgranulopoiesis: &50% of all segmented neutrophils have no granules or very few granules, or show the Pelger anomaly, or are peroxidase-negative. Criteria for dyserythropoiesis: &50% of the red cell precursor cells display one of the following anomalies: karyorrhexis, megaloblastoid traits, more than one nucleus, nuclear fragmentation. Criteria for dysmegakaryopoiesis: &50% of at least six megakaryocytes show one of the following anomalies: micromegakaryocytes, more than one separate nucleus, large mononuclear cells. In such a case, a very detailed analysis is essential for a differential diagnosis versus aplastic anemia (see pp. Common attributes are a usually slightly smaller cell nucleus and denser chromatin structure, the grainy consistency of which can be made out only with optimal smear technique. Advanced diagnostics start with peroxidase and esterase tests on fresh smears, performed in a hematology laboratory, together with (as a minimum) immunological marker studies carried out on fresh heparinized blood samples in a specialist laboratory. The cells in acute lymphocytic leukemia vary, and the subtypes can be reliably identified only by immunological methods a b c d. The transitions between the forms of myelodysplastic syndromes are very fluid, and they have the following features in common: Anemia, bicytopenia, or tricytopenia without known cause. In unexplained anemia and/or leukocytopenia and/or thrombocytopenia, blood cell abnormalities may indicate myelodysplasia a b c d e. The cytoplasmic hypogranulation is also observed in normal segmented granulocytes. These abnormalities are seen in myelodysplasia or after chemotherapy, among other conditions. A macrocytic anemia, the 5qsyndrome manifests with normal or increased thrombocyte counts while the bone marrow contains megakaryocytes with hyposegmented round nuclei. Naturally, bone marrow analysis is of particular importance in the myelodysplasias. The classification of myelodysplasias requires bone marrow analysis a b c d. Relatively small disk-forming megakaryocytes (1) and multiple singular nuclei (2) are often seen. Particularly striking is the coarse nuclear structure with very light gaps in the chromatin (arrow 1). Leukocytosis with a predominance of segmented neutrophilic granulocytes without the less mature forms is called granulocytosis or neutrophilia. Prevalence of Polynuclear (Segmented) Cells 111 Causes of Neutrophilia All kinds of stress Pregnancy Connective tissue diseases Tissue necrosis. This left shift almost always indicates an increase in new cell production in this cell series. However, since total leukocyte counts are subject to various interfering factors that can also alter the cell distribution, left shift without leukocytosis can occur, and has no further diagnostic value. At best, if no other explanations offer, a left shift without leukemia can prompt investigation for splenomegaly, which would have prevented elevation of the leukocyte count by increased sequestration of leukocytes as in hypersplenism. In evaluating the magnitude of a left shift, the basic principle is that the more immature the cell forms, the more rarely they appear; and that there is a continuum starting from segmented granulocytes and sometimes reaching as far as myeloblasts. Accordingly, a moderate left shift of medium magnitude may include myelocytes and a severe left shift may go as far as a few promyelocytes and (very rarely) myeloblasts, all depending on how fulminant the triggering process is and how responsive the individual. The term “pathological left shift” (for a left shift that includes promyelocytes and myeloblasts) is inappropriate, because such observations can reflect a very active physiological reaction, perhaps following a “leukemoid reaction” with pronounced left shift and leukocytosis. Causes of Reactive Left Shift Left shift occurs regularly in the following situations: Bacterial infections (including miliary tuberculosis), Nonbacterial inflammation. Predominance of the granulocytic lineage with copious granulation and sporadic immature cells: usually a reactive left shift a b d c e. Particularly striking are the reddish granules in a band neutrophilic granulocyte (2). The chronic myeloproliferative disorders encompass chronic autonomous disorders of the bone marrow and the embryonic blood-generating organs (spleen and liver), which may involve one or several cell lines. The common attributes of these diseases are onset in middle age, development of splenomegaly, and slow disease progression (Table 20). No No No chromosome Other chroIn the acceldel (20q), +8, -7, +8, +9, Very rare mosomal eration phase +9, +1q, and +1q, and others alterations and blast criothers sis Alkaline ""! Normal Normal # 900 pg/ml Theml, Color Atlas of Hematology © 2004 Thieme All rights reserved. Clinical findings: Slowly developing fatigue, anemia; in some cases palpable splenomegaly; no fever. The average “typical” cell composition is as follows (in a series analyzed by Spiers): about 2% myeloblasts, 3% promyelocytes, 24% myelocytes, 8% metamyelocytes, 57% band and segmented neutrophilic granulocytes, 3% basophils, 2% eosinophils, 3% lymphocytes, and 1% monocytes. Analysis of the bone marrow should be performed because it provides a series of insights into the disease processes. Histiocytes may store glucocerebrosides, as in Gaucher syndrome (pseudo-Gaucher cells), or lipids in the form of sea-blue precipitates (sea-blue histiocytes after Romanowsky staining). Megakaryocytes are usually increased and are often present as micromegakaryocytes, with one or two nuclei which are only slightly larger than those of promyelocytes. Their cytoplasm typically shows clouds of granules, as in the maturation of thrombocytes. Bone marrow analysis is not obligatory in chronic myeloid leukemia, but helps to distinguish between the various chronic myeloproliferative disorders a b c. When the blast and promyelocyte fractions together make up 30%, and at the same time Hb has decreased to less than 10g/dl and the thrombocyte count is less than 100000/µl, an incipient acute blast crisis must be assumed.

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