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A man aged 57 years with a chronic megakaryocytic granulocytic myelosis, treated with an intermittent low-dose busulfan schedule, developed intrahepatic cholestasis. We advise to do liver biopsy, if possible, to differentiate between busulfan hepatotoxicity and leukaemic activity. In the former case, it may be necessary to stop busulfan.
Bone marrow necrosis (BMN) is a necrosis of the hemopoietic tissue including the fibrovascular medullary stroma. Most frequently, it is caused by failure of bone marrow microcirculation. It is a complication in a wide spectrum of diseases, most frequently of malignancies, and is only rarely diagnosed ante mortem. In 6 of our 7 intravitally diagnosed cases, BMN was recognized already at the cytological examination of the bone marrow and was verified by the histological examination of the biopsy specimens as well as at necropsy. All our patients suffered from various malignant diseases. Three had generalized gastric carcinoma, the remaining hematological neoplasias: Acute lymphoblastic leukemia, acute monocytic leukemia, blastic transformation of chronic granulomegakaryocytic myelosis and primary medullary centrocytic lymphoma. The survival varied from 4 to 14 weeks after the BMN diagnosis. Clinical, hematological and autopsy findings as well as the etiopathogenetic views and prognostic implications of the diagnosis are discussed.
For 147 patients with myeloproliferative diseases, a study was made of the expression of Fc-receptors to immunoglobulins IgC, IgA, and that of FcH-receptor and receptors to C3-components of the complement in peripheral blood neutrophils. The data obtained show the lower level of neutrophils having membrane receptors in patients with acute myeloblastic leukemia and chronic granulocytic leukemia at the stage of blast transformation. A decrease in expression of membrane receptors of neutrophils was shown in patients with chronic granulocytic leukemia and benign subleukemic myelosis. The finding of a higher level of neutrophils having surface receptors revealed in patients with real polycythemia is close to the data obtained in the study of expression of membrane receptors in patients with chronic myeloproliferative diseases and healthy persons.
Pararheumatic complaints may be the leading clinical symptom in the individual case also in leucoses and may lead to diagnostic errors. They are caused by leucotic infiltrates in juxta-articular bone segments, sporadically also by blast infiltrates of the synovial membrane, by secondary osteoporoses or osteoscleroses and by articular haemorrhages. In altogether 254 patients with leucosis there were rheumatoid symptoms in 21% of the cases with undifferentiated cell leucoses, in 37% of the cases with chronic myelosis, and in 12% of the cases with chronic lymphadenosis. Diagnostic errors are deplorable above all in the undifferentiated cell leucosis, as every temporary retardation of the beginning of the therapy furthermore deteriorated the prognosis of the disease.
One thousand and five bone marrow biopsies were performed in patients with haematologic or oncologic disorders during a ten year period from 1976 to 1985 according to the method of Jamshidi and Swaim. Indications and method of biopsy are discussed in detail. Major side effects were not observed, however minor accidents (0.2%) as well as problems in yielding biopsy-material (1.6%) are reported. The rate of biopsy-failure, including biopsies with insufficient (crushed) material, was 5%. In our hands the predominant value of the Jamshidi-biopsy for diagnosis of hematologic disorders is given by the following reasons: Bone marrow histology gives a more detailed architectural picture than cytologic smears. Sampling of bone marrow for both methods (cytology and histology) through the same instrument is possible. The procedure is easily performed and gives the patient no more discomfort than a simple sternal puncture. Chronic myeloproliferative disorders (CMPD, 31%), malignant lymphomas (40%) and aplastic (hypoplastic) syndromes (4%) were the most frequent indications for bone marrow biopsy. Clinical and histological findings were compared in 235 patients with CMPD. The histological defined entity of chronic megacaryocytic-granulocytic myelosis could be differentiated easily from chronic granulocytic leukemia (CGL), however it was not always distinguishable from primary thrombocythemia by means of clinical and hematological criteria. Myelofibroses on the basis of CGL were separated from idiopathic or postpolycythemic fibroses by hematological findings. The diagnostic value of bone marrow biopsies was superior to cytology in all CMPD and proved to be an essential diagnostic method in cases with high platelet count. Marrow involvement was found in 59% of 218 previously untreated patients with non Hodgkin's lymphomas and in 9% of 123 patients with Hodgkin's disease. Jamshidi-biopsy proved to be a simple and indispensable procedure in staging of Hodgkin's and non-Hodgkin's lymphomas.
The populations of lymphocytes and serum immunoglobulins were explored in 20 patients with erythremia, 26 patients with sub-leukemic myelosis, 38 patients with pronounced chronic myeloid leukemia, and 33 patients with the blastic phase of the illness. The patients with chronic myeloproliferative diseases manifested cellular immunodeficiency that was aggravated as the disease progressed. In the pronounced stage, the patients showed relative preservation of humoral immunity whereas in the terminal stage they developed humoral deficiency. Study of the time-course of changes in the immunological characteristics has demonstrated that deterioration of the characteristics starts during blast transformation of myeloproliferative diseases. This may serve as a criterion for predicting exacerbations and early diagnosis of the blastic phase.
Plasma fibronectin is regarded to play an important part in a decrease of the resistance to infections. To specify the role of fibronectin in the pathogenesis of infectious complications in patients with depressions of hemopoiesis, the content of this opsonin was measured by ELISA in 113 patients with different patterns of hemoblastoses, lymphoproliferative diseases and with an aplastic syndrome. In 42 patients, the concentration of opsonin was measured in the presence of the superimposed infection of varying gravity. The fibronectin content was examined in 39 patients before, during and after completion of the cytostatic polychemotherapy. It turned out that in patients with paraproteinemic hemoblastoses, lymphogranulomatosis, aplastic anemia, chronic lympholeukemia, acute lympho- and myelo(mono)blastic leukemias, cyclic neutropenia, chronic myelosis and hematosarcomas, the concentration of fibronectin remained normal in the absence of infections. The computation of the linear correlation ratio did not reveal any association between the opsonin level and the concentration of neoplastic elements in the peripheral blood. Repeated measurements of the fibronectin level in patients whose underlying disease ran its course in association with marked neoplastic fever failed to detect any deficiency of the glycoprotein. The lowering of the fibronectin level was recorded in patients with a grave concomitant infection of the type of sepsis, necrotic enteropathy and lobar pneumonia. The degree of opsonin deficiency correlated with the patients' disease gravity. Prolonged reduction in the blood fibronectin level was of unfavourable prognostic importance. Cytostatic polychemotherapy, myelotoxic agranulocytosis as well as infectious complications of low gravity did not influence the concentration of fibronectin.(ABSTRACT TRUNCATED AT 250 WORDS)
A case of idiopathic autoimmune hemolytic anemia with subacute onset and peculiar course is reported. In the beginning of the disease there was a long lasting severe aplastic phase, during which numerous blood transfusions were required. The low antiglobuline test titre compared with the severe hemolytic activity and the ineffective erythropoesis suggested first of all the presence of pure red cell anemia. Later on blood regeneration with erythroblastosis started and resembled the picture of erythremic myelosis. The transient course of the disease with full normalisation of all blood values and continuous remission lasting now for more than one year is a peculiar variant of idiopathic autoimmune hemolytic anemia.
Fine structure of lymphoblastoid cell lines (LCL) derived from leukocytes of three patients with acute myelosis and one patient with undifferentiated acute leukemia were studied repeatedly by electron microscope. The majority of cells in all lines was of the same primitive lymphoblastic type as refered to in literature regardless to their origin. Morphology of the cells did not change with time of cultivation for several months, by freezing and thawing and by new recultivation after prolonged cryopreservation. No morphologic signs of contamination by viruses were found. No evident morphologic signs of differentiation to mature granulocytes or "mature" lymphocytes or plasmatic cells were observed.
Neurological complications in the light of their clinical and topographical pattern are discussed with regard to the literature and 40 personal cases. Peripheral neuropathy is the most common (average frequency 26%). The main clinical, anatomical, histological and pathogenetic features of polyneuritis in diabetes are illustrated. Diabetic amyotrophy is a true clinical entity, though its site (neural or muscular) and pathogenesis are still the subject of discussion. Cranial nerve damage (oculomotor paralysis in particular) has the typical clinical, anatomical and histological picture of peripheral forms. Myelopathy leads to three distinct anatomical and clinical patterns: pseudo-tabes caused by degeneration of the roots and posterior cords; chronic anterior poliomyelitis due to degeneration of the cells of the anterior cornua; myelosis attributable to combined degeneration of the posterior and anterolateral cords. The main features of encephalopathy and the relation between epilepsy and diabetes are also examined.
The chronic myeloproliferative disorders (MPD) are well characterized clinical entities comprising polycythaemia vera (PV), idiopathic thrombocythaemia (IT) (also called megakaryocytic myelosis, mature type; MegM), chronic myeloid leukaemia (CML), and myelofibrosis/osteomyelosclerosis (MF/OMS). Three thousand one hundred and eight bone biopsies of 2629 patients with established MPD were examined to investigate the histologic features of MPD in a large material in order to identify criteria for the histologic classification and differential diagnosis of these disorders. Detailed histologic characteristics were defined for each of the disorders and the results showed that in the majority of cases MPD may be recognized and classified by the initial bone marrow histology. Utilizing the predominant proliferative cell(s) in the bone marrow, PV was categorized into 4 types: 1. the classic, tri-linear type; 2. a bi-linear type with hyperplasia of the erythroid and granulocytic lines; 3. a bi-linear type with hyperplasia of the erythroid and megakaryocytic cell lines and 4. a uni-linear type with isolated increased erythrocytic proliferation. CML showed 2 sub-divisions: 1. the granulocytic, uni-linear type and 2. the bi-linear type with proliferation of myeloid and megakaryocytic lines. The former had a tendency to evolve into blastic crisis, while the latter was prone to develop into MF/OMS. It was primarily uni-linear exhibiting increased megakaryocytes. In most cases, MF/OMS was shown (by means of follow-up biopsies) to arise out of those entities of MPD which had included megakaryocytic hyperplasia and to which the proliferation of fibroblasts was secondary. The conclusion is drawn that an initial bone marrow biopsy provides additional diagnostic and prognostic data in this group of haematologic malignancies.
Morphometry was employed on different entities of chronic myeloproliferative diseases (CMPD) and reactive lesions in addition to normal control specimens. The entities studied included: (1) inflammatory reactions of the bone marrow (so-called myelitis in chronic rheumatoid arthritis), (2) chronic granulocytic leukemia (CGL), (3) agnogenic myeloid metaplasia in an early hypercellular stage (so-called chronic megakaryocytic-granulocytic myelosis, CMGM), (4) agnogenic myeloid metaplasia in an advanced fibrosclerotic stage or osteomyelofibrosis/sclerosis (MF/OMS), (5) polycythemia vera (P. vera), (6) reactive thrombocytosis (TH, as a sequel of miscellaneous conditions) and (7) primary (idiopathic, essential) thrombocythemia (PTH). Evaluation was done on plastic-embedded semithin sections with a constant thickness of 3 micron in approximately 20 cases of each group of CMPD. The following parameters were determined: (1) density distributions of the megakaryocyte and non-megakaryocyte compartments, (2) arrangement of megakaryopoiesis in the bone marrow space (i.e., inhomogeneity or clustering) and (3) the fine structure of megakaryocytes in PTH, with a quantitative analysis of the nuclear morphology by circular deviation and contour factors. The megakaryocyte morphology was closely related to a facultative or obligatory increase of the platelet count in these various entities of CMPD and was separable into two major categories: (1) controls, CGL and myelitis versus (2) CMGM, MF/OMS, P. vera, TH and PTH. These two categories were distinguishable by the prominence of megakaryopoiesis in the bone marrow as well as the elevated platelet counts in the periphery. Moreover, in comparison with CMGM and MF/OMS, PTH was characterized by an apparently normal maturation and a conspicuous polyploidization of megakaryocytes according to the nuclear morphology, which was similar to that of P. vera. Our results suggest that PTH presents a monolinear growth of the megakaryopoiesis in the same way as CGL exhibits a monolinear proliferation of the neutrophilic granulopoiesis. This is in contrast to the mixed cellularity of both the megakaryocyte and granulocyte lineage in CMGM and MF/OMS.
Division of myeloproliferative syndrome is recommended based on 195 observer cases: A) Secondary myeloproliferative syndrome usually accompanies marrow carcinosis. It is characterized by non-destructive embryonal-type myeloproliferation occurring even outside the marrow excepting lymph nodes as a rule. There are neither specific changes in karyogram nor in alkaline phosphatase positivity. B) Idiopathic myeloproliferative syndrome is characterized (in comparison to A) by dysplastic changes especially in megakaryocytic line; it develops slowly tending to malignancy, namely leukemia or erythroleukemia that keep (unlike spontaneous leukemia) more severe dysplastic changes of megakaryocytes. Alkaline phosphatase is increased, atypical karyogram is not changed in Ph 1 region. C) Malignant neoplastic myeloproliferation of panmyelosis type is a primary destructive process akin to myelosis. Alkaline phosphatase is decreased, there are typical Ph 1 changes in karyogram and tumorous lymph node infiltration. Secondary myeloproliferative syndrome follows rarely and a mixed picture can be observed then, of course without severe megakaryocytic dysplasia. D) Myelofibrosis is an uncharacteristic final picture of various origin which neither develops in myeloproliferative syndrome or substitutionary extramedullar hemopoesis. Hesitation in oncological typing of idiopathic myeloproliferative syndrome cannot influence its nosological individuality. Exceptional and unexpected positive markers (alkaline phosphatase, Ph 1) occur in diagnostical practice from time to time; being unexplained they hinder from precise typing.
A patient with myeloproliferative syndrome presenting the variety of aspects of both polycythemia vera and agnogenic myeloid metaplasia and megakaryocytic myelosis is described. According to the more recent hypotheses on the matter, our report confirms the presence of inseparable links among the above mentioned myeloproliferative disorders. The wrong diagnosis of Hodgkin's disease based on the histological features of the spleen is also discussed.
Six of nine decedent beagles exposed continuously to 2.5 R*/22 hour day of whole-body 60Co gamma-radiation died with myeloproliferative diseases: three cases of myelogenous leukemia and one each of monocytic leukemia, erythroleukemia, and erythremic myelosis. The three dogs that died with myelogenous leukemia had micromegakaryocytes and megakaryoblasts in the peripheral blood during the preleukemic phase when myeloblasts were not observed in the peripheral blood or in increased numbers in the bone marrow. In this study we have examined the megakaryocytes during the preleukemic period by a combination of light, transmission, and scanning electron microscopy. Morphologic abnormalities seen by light microscopy included mononucleated and binucleated forms, many with cytoplasmic blebs. The small mononuclear forms in the bone marrow tended to form clusters. Ultrastructural features included a paucity of both specific alpha granules and dense granules. The micromegakaryocytes showed dysgenesis of the demarcation membrane system. This membrane system appeared disorganized with a few dilated round, oval, or rarely, elongated vesicles and showed no evidence of platelet formation. The cells also had a paucity of endoplasmic reticulum, few mitochrondria, and sparse glycogen accumulations. The scarcity of cytoplasmic organelles gave a pale immature appearance to the cytoplasm. By scanning electron microscopy, the sponge-like surface of large mature megakaryocytes from unirradiated marrow contrasted with the characteristically smooth, topographically featureless surfaces of the micromegakaryocytes from preleukemic dogs.
On principle one has to distinguish between inflammatory (infectious) and non-inflammatory intestinal diseases. These may affect merely certain parts of the gastrointestinal tract or influence the entire digestive system. Leading symptoms of all these diseases are disturbances of intestinal motility and of secretion, both causing diarrhoea or, rarely, constipation. The clinical picture can be acute or chronic. Acute manifestations, especially with high temperatures, include various florid psychopathological phenomena of an exogenous type of reaction. Chronic diseases of the gastrointestinal system do not show the above-mentioned picture. Very rarely encephalopathy causes organic psychosyndromes. Neurological findings point to lesions of the peripheral nerves (polyneuropathy), funicular defects within the spinal cord (funicular myelitis) and, very rarely, to encephalopathy. Syndromes of polyneuritis can be seen in acute, as well as in chronic enteropathies. Spinal lesions are only found in cases with chronic gastro-intestinal disease. With the exception of funicular myelosis in genuine pernicious anaemia (here parenteral substitution with vitamin B 12 is the therapy of choice) -- all gastroenterogenous neurological signs are only minimally accounted for by disturbances of B 1 and B 12 absorption. These substances should be used only in combination with other agents combating the basic disease.
A family is described in which two sisters developed acute myeloblastic leukemia (AML FAB M2) and erythroleukemia (FAB M6) at ages 60 and 53, 15 years apart. Their father was diagnosed as having chronic erythremic myelosis (DiGuglielmo syndrome) 24 years earlier at age 56. Cytogenetic analysis of bone marrow blasts in the patient with AML M2 revealed a complex hyperdiploid karyotype with clonal abnormalities -3, -5, del(5)(q13q23), -8, +i(8)(q10), -11, add(11) (q23), add(12)(p13), -13, +3mar. No environmental risk factors could be identified. Hematologic and cytogenetic analysis of all living first degree relatives of the affected persons revealed hematological abnormalities, but normal constitutional karyotypes of peripheral blood lymphocytes and no induction of cytogenetic abnormalities in folate deficient medium.
Immune status in relation to tumor progression was evaluated in 175 patients with chronic myeloproliferative diseases. 113 of them had chronic myeloid leukemia, 62 chronic subleukemic myelosis. It was established that immunologic reactivity decline correlated with the rate of tumor progression. The degree of immune deficiency should be taken into consideration in selection of patients for immunotherapy.