A 4 6/12-year-old boy presented with severe hemolytic uremic syndrome (HUS) and then developed acute lymphocytic leukemia (ALL) 8 months later. Although other syndromes of hematopoietic dysfunction have been reported to precede the overt and classic findings of leukemia, this report describes a patient who developed ALL preceded by HUS, ...

Gastrointestinal bleeding from Meckel's diverticulum resulted in small bowel obstruction by thrombus in two patients with acute myelogenous leukemia during bone marrow aplasia and recovery from induction chemotherapy. Although gastrointestinal symptoms and complications are common in acute leukemia, these two cases are unique and describe a new syndrome that requires ...

Gingival hyperplasia in a patient with myelodysplastic syndrome is described. Gingival infiltration was the first sign of acceleration of a stable disease process and was followed by development of a more aggressive phase of chronic myelomonocytic leukemia that was not responsive to therapy. Oral and dental assessment of patients with ...

An unusual myelodysplastic syndrome with similar features in two patients is described. The entity is characterized by a maturation arrest at the myelocyte stage, strikingly clumpy chromatin, and a clinical course marked primarily by difficulties caused by anemia and thrombocytopenia. Electron microscopic description of the characteristic abnormal clumpy chromatin cells ...

Specific cutaneous infiltrates in hairy-cell leukemia have distinctive histopathologic and histochemical findings. The true incidence of leukemia cutis in hairy-cell leukemia is uncertain, however, because reports often fail to document these distinctive features. A case of leukemic macrocheilitis associated with hairy-cell leukemia and the Melkersson-Rosenthal syndrome is presented. The Melkersson-Rosenthal ...

Hepatorenal syndrome and portal hypertension developed in a 59-year-old man with chronic lymphocytic leukemia. At autopsy, he had portal hypertension from lymphocytic infiltration of the liver with compression of the portal veins, and no other pathologic process in the liver. Histologic examination of the kidney did not reveal a lesion ...

Thirty-five years ago, a handful of astute clinical hematologists began to notice that some of their patients with acute nonlymphocytic leukemia had a history of a preceding ill-defined hemopathy. This "preleukemic" hemopathy was increasingly reported anecdotally and through careful retrospective analyses. In more recent prospective studies, this syndrome has been ...

Four patients with acute nonlymphocytic leukemia developed one or more components of the "ara-C syndrome" including fever, peritonitis, pericarditis, and a maculopapular rash during therapy with continuous infusions of low-dose (20 mg/m2/d) cytosine arabinoside (ara-C). These complications, described with standard and high doses of ara-C, have not been previously noted ...

The interpretation of small numbers of lymphoblasts or of nonspecific pleocytosis in the cerebrospinal fluid (CSF) of children with lymphoid malignancies is difficult. In this prospective study of 204 patients, 15 had white cell chamber counts of less than or equal to 10 cells/microliter, with one or more blasts on ...

In 98 patients with chronic myeloproliferative disorders (45 chr. myeloid leukaemia (CML), 19 myelofibrosis primaria (MP), 28 polycythaemia vera (PV) and 6 idiopathic thrombocythaemia (IT)) the incidences of increased numbers of MPO-deficient polymorphonuclear (PMN) were 60% in CML, 32% in MP, 7% in PV and 0% in IT patients. The ...

A 47-year-old man with hyperleukocytic chronic lymphocytic leukemia progressively developed retinal hemorrhages, headache, diplopia, dysequilibrium, slurred speech, nystagmus, ataxic gait, and hearing loss as his leukocyte count rose to a maximum of 968,000/mm3. All of these symptoms and signs resolved promptly after leukapheresis. The authors reviewed records of 210 patients ...

A 6-year-old girl with sickle cell disease was admitted to the hospital with the diagnosis of the acute chest syndrome. The laboratory findings and the radionuclear lung scan supported a diagnosis of pulmonary infarction rather than pneumonia. She improved with intravenous fluids, oxygen, penicillin, and theophylline. The most likely explanation ...

A girl with Down's syndrome was born with a myeloproliferative disorder. The child had spontaneous regression of the myeloproliferation, with acute leukemia developing at a later date. Morphologic, cytochemical, immunologic, and immunoglobulin gene configuration studies all supported the diagnosis of acute nonlymphocytic leukemia. High-resolution chromosome studies revealed that the leukemic ...

Using chimeric murine leukemia viruses (MuLVs) constructed in vitro with parental viral genomes from the neurotropic Cas-BR-E MuLV and the nonneurotropic amphotropic 4070-A MuLV, we previously mapped the paralysis-inducing determinant of Cas-BR-E MuLV within a pol-env region. To assess the role of the long terminal repeats (LTRs) in influencing the ...

Two children are described with acute leukemia in remission who both developed a swinging pyrexia and pathologically identical splenic necrotizing granulomata. No organisms were seen in, or isolated from, either spleen. After splenectomy, systemic symptoms persisted and did not resolve until steroids were given. The etiology of this syndrome remains ...

Of the myeloproliferative disorders which develop in Down's syndrome, acute leukemia associated with trisomy 8 has distinct characteristics. It is non-lymphoblastic in type and has a preleukemia phase in which thrombocytopenia is a prominent feature. One case occurring in a 20 mth-old girl is reported and 3 other similar cases ...

The author describes the phenomenology and clinical characteristics of survival guilt which can be found in many Vietnam veterans. This conflict, in its most acute presentation, typically resembles an agitated depression. The syndrome is characterized by the veterans' frequent dreams of their friends dying in battle, and by their avoidance ...

Two patients with Cogan's syndrome had acute bilateral hearing loss and vestibular dysfunction. Interstitial keratitis was discovered by slit-lamp examination. Both cases responded to corticosteroid treatment. Immediate recognition of this syndrome and the institution of high-dose corticosteroid therapy should lead to resolution of the hearing loss in patients with Cogan's ...

The authors report a documented case of hyperphosphatemia-induced nephrocalcinosis after chemotherapy of an acute lymphoblastic leukemia (ALL). Microscopic examination of the kidney showed numerous calcium deposits with concentric structure in the calyces and in the tubules. Electronic microsound analysis proved the deposits to be composed of calcium phosphate. The nephropathy ...

Phycomycosis is an opportunistic infection occurring in immunocompromised hosts. It is the most acutely fatal fungal disease known. Therefore, early recognition and treatment are essential. Ocular and orbital involvement is part of a rhino-orbital-cerebral form of the disease. The orbital apex syndrome is the hallmark orbital involvement. A case of ...

The 5q- syndrome is a recently described entity characterized by partial deletion of the long arm of chromosome No. 5 and by hematologic findings of chronic anemia with reticulocytopenia, nonlobulated megakaryocytes, and megathrombocytes. We report on a patient with the hematologic features of the 5q- syndrome who progressed to acute ...

A 55-year-old woman with an aortic arch syndrome of acute onset and dysphasia, resulting from the excessive administration of ergotamine tartrate suppositories, is described. Complete resolution of symptoms and return of upper limb and carotid artery pulses to normal occurred within four days of cessation of the ergot derivative. Lower ...

Tn polyagglutination or persistent mixed field polyagglutination is a rare acquired erythrocytic state which has been associated with haematologic abnormalities including four reported cases of acute leukaemia. Recent information has proven that the abnormality is clonal and the haematologic findings suggest a mutation of a somatic stem cell. The red ...

Between 1971 and 1981 756 children were diagnosed to have leukaemia in Hungary. Of these, 17 had Down's syndrome. Analyzing the clinical course of their leukaemia revealed a low remission rate and very poor survival in these patients. Down's syndrome seems to present a special high-risk feature, for early death ...

Nine patients with acute leukaemia developed a complex syndrome of hypocalcaemia and hypomagnesaemia during treatment. These metabolic abnormalities developed rapidly in some patients and gave rise to acute symptoms. Immunoreactive parathormone concentrations in these patients were found to be either absolutely or inappropriately low. Hypomagnesaemia and the action of chemotherapeutic ...

The Sézary syndrome was diagnosed in a 71-year-old black woman with erythroderma, generalized lymphadenopathy and hepatosplenomegaly. The laboratory data revealed a white blood cell count of 65,000 mm3 with 81% lymphocytes, the majority having an indented or a cerebriform nucleus. The skin biopsy, the lymph node biopsy and immunologic surface ...

In five out of 51 adult, male patients with acute non-lymphocytic leukemia, Klinefelter's syndrome was demonstrated karyotypically. The incidence of this syndrome in our material is very much increased in comparison with the frequency at birth. In this article the medical histories of the patients are given and the possible ...

Acute megakaryoblastic leukemia or acute "malignant" myelosclerosis is an acute and rapidly progressive myeloproliferative syndrome characterized by minimal or absent splenomegaly, pancytopenia, diffuse marrow fibrosis, and circulating blasts of megakaryocytic origin. The disease must be differentiated from other hematologic malignancies especially myelofibrosis with myeloid metaplasia. The radiographic changes of osteosclerosis ...

A 29-year-old female with acute promyelocytic leukemia presented with severe liver dysfunction and disseminated intravascular coagulation. Her condition rapidly deteriorated and she expired before any antileukemic therapy could be administered. At autopsy, she was found to have massive thrombosis of hepatic veins and their tributaries. Microscopic picture of liver was ...

Idiopathic myelofibrosis (IM) is a myeloproliferative syndrome characterized by anemia, fibrosis of the bone marrow, variable proliferation of megakaryocytes, myelocytic and erythrocytic precursors, and splenomegaly. Extramedullary hematopoiesis can develop in diverse areas. Unlike in chronic granulocytic leukemia, transformation to acute leukemia is uncommon in IM. Transformation to acute leukemia is ...

Bone marrow and peripheral blood cells from three newborns with Down's syndrome and transient myeloproliferative disorders were cultured in vitro. In the methylcellulose semiliquid system, normal colony formation with maturation and differentiation into granulocytes and monocyte-macrophages were observed in all three patients. This is different from the growth pattern usually ...

A rare syndrome of acute pulmonary damage due to gold therapy is described. It appears to be an entity separate from the usually reported side-effects of gold. It has a good prognosis, responding to the cessation of gold and to corticosteroid therapy. Apart from this syndrome of acute pulmonary damage, ...

A 10-year-old boy with leukemia developed ipsilateral corticospinal and cerebellar signs: the ataxic hemiparesis syndrome. Computed tomography revealed a mass lesion in the contralateral rostral midbrain. Previous reports of this syndrome placed the lesion in the basis pontis. The anatomical substrate of the ataxic hemiparesis syndrome includes the basis pontis, ...

Four patients with non-Hodgkin lymphoma and two with acute lymphocytic leukemia (ages 4 and 4 months to 16 years 6 months) exhibited a unique reaction to intravenously administered cytosine arabinoside (Ara-C) given alone as a part of the previously reported LSA2-L2 treatment protocol. The syndrome was characterized by fever, myalgia, ...

Two women with hematological and clinical features typical of the preleukemic syndrome, including low marrow myeloid colony and cluster formation in vitro, showed unexplained spontaneous improvement with normalization of both peripheral blood values and in vitro cloning efficiency after 2-4 years. One patient had and the other developed later an ...

Three patients with systemic T-cell leukemia-lymphoma presented with generalized pruritic skin rash which was clinically nondiagnostic. Biopsies revealed infiltration of the superficial dermis by atypical lymphoid cells with epidermal exocytosis (Pautrier abscesses) producing a pattern of histologic involvement indistinguishable from that of mycosis fungoides and Sézary syndrome. This striking similarity ...

A serial clinical, hematologic, and cytogenetic study was done on a baby with Down's syndrome in whom a myeloid leukemoid reaction developed at birth that spontaneously regressed within a month only to relapse two years later to an acute undifferentiated stem cell leukemia. He died 1 1/2 months after onset. ...

The preleukemic syndrome or hematopoietic dysplasia is a marrow stem-cell disorder with clinically recognizable hematologic abnormalities which precede the development of acute nonlymphocytic leukemia. Its occurrence in childhood is extremely rare; seven "true" cases who fulfill all the criteria for the disorder have been reported until now. The preleukemic syndrome ...

Three cases of acute non-promyelocytic leukemia complicated with intravascular coagulation and fibrinolysis syndrome were described. The blood clotting system and fibrinolysis studies in all 3 cases revealed a significant increase of FDP level and shortening of fibrinolysis time as well as a decrease of fibrinogen content in 2 cases. In ...

The clinical and morphological characteristics of Shwachman's syndrome (exocrine pancreatic insufficiency, pancytopenia, skeletal changes) were observed in a boy who, at the age of 8 years, developed a juvenile form of chronic myeloic leukemia which did not respond to cytostatic treatment. Autopsy revealed a striking lipomatous atrophy of the pancreas, ...

The study presents the clinical symptomatology of acute embolic-toxic reactions to penicillin seen in 9 patients treated with the drug. The complex of short-lasting symptoms characterized by severe agitation with confusion, visual and auditory hallucinations and dire fear is caused by accidental penetration of an amount of intramuscular penicillin injected ...

A 5 1/2-year-old child with Bloom's syndrome developed acute lymphocytic leukemia (ALL). Bloom's syndrome is associated with chromosomal aberrations, and affected individuals have an increased incidence of leukemia and solid tumors. The skin on our patient had adjacent areas of decreased and increased pigmentation similar to the "twin-spots" seen in ...

A patient under treatment for chronic lymphocytic leukaemia developed lobar pneumonia after 8 months. When antileukaemic therapy was discontinued, features of an ectopic ACTH syndrome developed, secondary to bronchogenic carcinoma. Exogenous steroid therapy for leukaemia seemed to suppress the clinical manifestations of the ectopic ACTH syndrome while subsequent endogenous steroid ...

A 64-year-old hypertensive man presented with the dysarthria--clumsy hand syndrome, manifested by dysarthria, dysphagia, central facial weakness, deviation of the tongue on protrusion, incoordination of the affected hand, and mild imbalance on walking. A computed tomograpphic scan demonstrated a resolving acute infarction in the vicinity of the genu of the ...

The rapid appearance of acute respiratory distress during the course of 25 hyperleukocytic leukemias was associated with the rapid increase of the leukocytosis. The regression of the tachypnea was spectacular when treating hyperleukocytosis by exchange transfusion and chemotherapy. Blood gas studies, although blurred to some extent by in vitro blast ...

This is the first report of a case of chronic myeloid leukaemia (CML) complicated by myasthenic syndrome. The patient suffered two prolonged periods of idiosyncratic busulphan-induced marrow aplasia, the first occurring 5 months after busulphan was stopped. Between these two episodes, and at a time when no therapy was required ...

Congenital leukaemia is a rare disease with approximately 100 cases reported in the literature. It is most often diagnosed as acute myelogenous leukaemia (AML). Leukaemic skin nodules and hepatosplenomegaly are the most frequent clinical findings noted. The laboratory manifestations include a markedly elevated white count with a large percentage of ...

A patient with acute lymphoblastic leukemia had an unusual prodrome, including clinical features of "hypereosinophilic syndrome," pulmonary infiltrates, and bilateral spontaneous pneumothorax, which preceded the onset of leukemia by four months. The mechanism for the production of eosinophilia may have been related to the production of eosinopoietic factors by the ...

Retrospective and prospective observations have established the existence of a recognizable hematologic syndrome preceding the development of acute nonlymphocytic leukemia. This syndrome, which has been termed the "preleukemic syndrome" or "hemopoietic dysplasia", appears to be a stage in a multiphasic myeloproliferative disorder, rather than a separable disease with a propensity ...

A 56-year-old man with chronic lymphocytic leukemia who developed Richter's syndrome is described. The criteria for diagnosis are given and the histology is discussed. We agree that Richter's syndrome represents a peculiar complication of chronic lymphocytic leukemia and not a separate disease entity. As such, Richter's syndrome must be known ...