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A case of myelofibrosis with papulonodular cutaneous lesions in a 49 year old man is reported. The histopathological studies showed the presence of the three hematopoietic lines in the skin lesions, which is a uncommon feature.
There is an extensive descriptive literature concerning both the clinical and pathologic features of myelofibrosis as it occurs in the setting of myeloproliferative disease. These initial chapters of this book have reviewed these aspects of the problem, and will therefore serve as a background against which recent new advances in the biology of collagen in particular, and connective tissue in general, can be viewed. These newer advances will constitute the remainder of this book.
A description of the clinical and haematological features of splenomegalic idiopathic myelofibrosis is followed by the presentation of two personal cases, one with a classic picture of the advanced stage and marked, progressive anaemia, leukopenia, frank splenomegaly and renal alterations, the second in the initial stage, with liver and spleen enlargement, very marked thrombocytosis and high white and red cell counts. The clinical diagnosis was confirmed by bone biopsy in each case. A description of the clinical findings precedes an explanation of the anatomical and histological pictures observed at biopsy. These are fundamental importance for the purposes of diagnosis.
Latex ingestion and nitroblue tetrazolium reduction were measured in neutrophils of 40 patients with primary idiopathic myelofibrosis. The percentage of neutrophils that ingested latex, in the presence of either autologous or control sera, was lower (P less than 0.001) than that of the controls. The percentage of the ingesting neutrophils that reduced nitroblue tetrazolium was also lower (P less than 0.001) in the patients than in the controls. Activities of glucose-6-phosphate dehydrogenase, 6-phosphogluconate dehydrogenase, glutathione peroxidase and NAD(P)H oxidases were not different from those of the controls. In contrast, glutathione reductase activity was significantly lower (P less than 0.001) in the patients than in the controls either with or without the addition of flavin adenine dinucleotide. Glutathione reductase and nitroblue tetrazolium reduction activities were correlated (r = 0.913). These results are discussed within the framework of the acquired enzymopathies and the increased susceptibility to infection observed in these patients.
The concomitant occurrence of non-Hodgkin's lymphoma and agnogenic myeloid metaplasia with myelofibrosis in one patient is described for the first time. Oral therapy with an alkylating agent resulted in partial remission.
Cutaneous manifestations of thrombocythaemia are caused by vascular lesions, either haemorrhagic or thrombotic, affecting above all the microcirculation of the extremities. The authors report a case of thrombocythaemia associated with myelofibrosis discovered by ischaemic lesions on the toes which appeared at the beginning of PUVA therapy for psoriasis. The type of vascular lesions generally depends on the platelet count. In the presence of these cutaneous manifestations, the determination of both platelet counts and functions may orient the diagnosis toward a myeloproliferative syndrome.
This paper reports the findings of an "acquired" hereditary angioedema-like syndrome in a patient with myelofibrosis. No previous personal or family history of angioedema was present. The serum complement pattern showed a marked reduction of Cl esterase inhibitor, Clq and C4. All family members had a normal complement profile. Because of frequent attacks of laryngeal angioedema, prophylactic treatment with danazol was started. A striking clinical response was observed as well as a normalizing effect on the underlying biochemical abnormality.
An FBP has been purified from the spleen (FBP-S) of a patient with agnogenic myeloid metaplasia and myelofibrosis. This binder is similar to a previously purified protein from CML cells (FBP-L) in its molecular weight and affinity for folate analogues. However, each protein has very little cross-reactivity in specific RIAs for each binder, indicating that there are structural differences which can be detected by the immune system. THe concentration of FBP-L in the serum from normal subjects was 2.0 +/- 0.39 ng/ml (mean +/- S.E.M.) and was significantly elevated in the serum from some patients with CML, uremia, hepatitis, and cancer. The concentration of FBP-S, on the other hand, was 8.31 +/- 0.51 ng/ml in normal serum and remained in this range in the same serum that contained the elevated concentration of FBP-L. In contrast, the concentration of FBP-L was only 13% to 30% of the concentration of FBP-S measured in the homogenates of normal and adjacent cancerous tissue from lungs and colons. These studies indicate that some FBP(s) in human tissues have immunologic specificity even though they are functionally similar.
Acute myelofibrosis (AMF) was diagnosed in a 59-yr-old black male in September 1978, on the basis of pancytopenia, lack of hepatosplenomegaly, fibrosis of the marrow, and paucity of teardrop red blood cells in the peripheral blood. Since then the patient has demonstrated an unusually long survival of 36 mo with a changing cytogenetic course. His initial 46, XY normal karyotype changed in 20 mo to trisomy 8, followed 1 yr later by 1:4 translocation in peripheral blood. Simultaneously with these changes, the fibrosis in the bone marrow progressively decreased, ultimately terminating in chronic granulocytic leukemia-like presentation with reversal to 46, XY karyotype. Fibroblast culture failed to show any evidence of cytogenetic abnormalities. The disappearance of fibrosis confirmed by trichrome and reticulin stains and lack of cytogenetic abnormalities in fibroblasts confirms the secondary role of fibrosis.
In 30 consecutive splenectomies for myelofibrosis (MF) with cytopenia(s) and/or massive splenomegaly the operative mortality was 6.7% (2 patients). Postoperative complications were observed in 11 (37%) patients. When surgery was undertaken within one year of the diagnosis of MF the postoperative morbidity rate was 13% (2/16), but significantly (p less than 0.01) higher at 64% (9/14) in patients operated on later. A similar significant difference was observed in the amount of intraoperative blood loss. Of the patients undergoing splenectomy for anaemia and/or thrombocytopenia, 79% gained definite benefit from the operation for 2 to 70 months (median 10 months). It is concluded that, although splenectomy probably does not prolong the life of patients with MF, it gives considerable symptomatic relief in cases with massive splenomegaly, and can improve the patient's haematological status and reduce blood transfusion requirements. To avoid the increased risks of postoperative complications surgery is best undertaken as soon as cytopenia is present.
We report on 15 patients affected by myelofibrosis. The main clinical aspects were hepatosplenomegaly, anemia and leukocytosis. Immature cells and anisopoikilocytosis of the erythrocytes were frequently found in the peripheral blood. At bone biopsy, various aspects of panhyperplasia, increase in the reticular fibers, myeloid fibrosis and osteosclerosis were observed. Leukocyte alkaline phosphatase was frequently elevated. The clinical and laboratory course of the disease, causes of death and the possible etiopathogenetic aspects of this disease are considered and discussed.
A 46-year-old man with primary myelofibrosis developed polyarthralgia. Marked hepatosplenomegaly was noted, and hematological examinations revealed a white cell count of 25,600/microliters with 42% promyelocytes and thrombocytopenia. The promyelocytes were positive for CD4 antigen and nonspecific esterase as well as peroxidase. Cytogenetic analysis of circulating mononuclear cells showed the trisomy of No. 22 chromosome in 3 of 5 cells examined. Four months later, the patient became asymptomatic, and hematological picture and hepatosplenomegaly returned to the original level. This is the first report describing the transient promyelocytic expansion in myeloproliferative disorders.
Primary myelofibrosis is a complex disorder characterized by bone marrow fibrosis with no apparent cause. It is known in literature under a wide number of terms, reflecting the variety of clinical features and the different pathogenetic hypotheses. In most cases it is plain that marrow fibrosis is secondary to a clonal myeloproliferative disorder and, in particular, to the presence of abnormal megakaryocytes secreting (MKDGF/PDGF); but probably some other growth factors synthesized by megakaryocytes and contained in platelet alpha-granules are involved. The molecular event that determines the advantage of the clonal growth is, at present, unknown, and the pathogenetic importance of some chromosome anomalies is still under discussion. Over the last years, besides megakaryocyte dysplasia, several fibrogenetic mechanisms such as a bone marrow immune damage have been taken into consideration. Studies on prognostic factors regarding the main clinical, hematological and histological parameters have given conflicting results, because of low incidence of the disease, different criteria used for the diagnosis, and different terms of the clinic presentation of the pathology. Although a great deal of progress has been made in terms of pathogenetic mechanisms, a lot of questions must be still definitively settled, further in depth studies still have to go into many matters.
We present a case of secondary myelofibrosis in which magnetic resonance imaging (MRI) contributed to the diagnosis. On both T1- and T2-weighted images, bone marrow showed hypointensity. However, since blood transfusion therapy was performed prior to MRI, it was necessary to differentiate fibrosis from siderotic marrow. On gradient echo (GRASS) images, bone marrow also showed hypointensity, but not as prominent as that seen in siderotic marrow. After intravenous administration of gadolinium-dimethylene triaminepentaacetic acid (Gd-DTPA), enhancement of the marrow space may be induced by a fibrous change of the marrow. MR findings were confirmed by histological examination of the biopsy specimen.
Functional potential of the kidneys, principal causes of nephrolithiasis and chronic renal failure were investigated in 30 idiopathic myelofibrosis (IMF) patients by combination of different modalities, sonography, in particular. Sonography proved highly informative in diagnosis of nephroliths the formation of which is attributed to derangement of uric acid metabolism, of renal elimination, and anatomical features of the urinary system. A correlation analysis between various factors showed that in IMF hyperuricemia, hyperfiltration and proteinuria damage cortical and medullary layers of the kidneys provoking their functional defects and development of chronic renal failure.
A patient who developed a low grade non-Hodgkin lymphoma two years after the diagnosis of an idiopathic myelofibrosis, is described. The pathogenic relationship between both processes is discussed, making a brief review of the literature.
We report a successful ereated case of acute megakaryoblastic leukemia (AMKL) with myelofibrosis (MF), which achieved a disease free condition, with disappearance of MF, for over 24 months after allogeneic bone marrow transplantation (BMT) and summarized cases of MF receiving BMT reported in Japan to evaluate the influence of MF on engraftment of bone marrow (BM). A 40-year-old man was admitted on Jan. 29, 1991 due to anemia and thrombocytopenia. BM aspiration resulted in a dry tap and MF and cells stained positive with anti-GPIIb/IIIa (CD41a) antibody were demonstrated by BM the biopsy specimen. Complete remission was achieved by multi-drug chemotherapy including behenoylcytosine arabinoside, etoposide, mitoxantrone and prednisolone (PLS). After preconditioning with little BU+CY, BMT was performed from an HLA-identical brother on Jan. 16, 1992. From day 9 of post BMT, acute skin graft versus host disease (grade 1) was observed, which was controlled by 60 mg/day of PSL. Engraftment was achieved on day 12. Although cystitis developed, he was discharged on Apr. 5, 1992 and remains disease free. Including the present case, seven allogeneic BMT patient with MF have been reported so far in Japan. Four cases in whom MF recovered before BMT showed better results than other three cases that still showed MF at BMT. Reversal of MF seems to be a favorable pre-transplant factor for successful BMT in patients with MF.
We report a splenoma associated with an important megakaryocyte sequestration in peliotic sinuses. The diagnosis was done on surgical specimen of splenectomy performed for a painful splenomegaly occurring in a 71-year-old man presenting a primary idiopathic myelofibrosis. Histopathological features of these splenic microvascularization disorders, mainly occurring during hemopathy involving the spleen, are discussed. This case seems to confirm the hypothesis that splenoma could be an acquired splenic disease.