Successful Splenectomy in May-Hegglin Anomaly: Report of a Case with Platelet Kinetic Studies

Raveh, David; Krausz, Yodphat; Eldor, A

doi:10.1159/000205277

Cited by 5 publications

(6 citation statements)

References 2 publications

(3 reference statements)

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“…Though splenectomy was successful in a case of macrothrombocytopenia with significant splenic sequestration, electron microscopy has not been performed in that study; thus the hematological disease might have been Fechtner syndrome as well [13]. Moreover, renal and auditory complications were present in several members of that family [13].…”

Section: Discussionmentioning

confidence: 89%

“…The bleeding tendency is mild and none of the renal or auditory complications characteristic of the other hereditary thrombocytopenias with giant platelets and inclusion bodies in the leukocytes (Fechtner syndrome, Sebastian platelet syndrome) are present [6,12]. Though splenectomy was successful in a case of macrothrombocytopenia with significant splenic sequestration, electron microscopy has not been performed in that study; thus the hematological disease might have been Fechtner syndrome as well [13]. Moreover, renal and auditory complications were present in several members of that family [13].…”

Section: Discussionmentioning

confidence: 97%

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Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

Demeter

Lelkes

Nemes

et al. 2001

Annals of Hematology

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We present two cases of the May-Hegglin anomaly discovered in a patient and one of her two sons. The female patient was known to have proteinuria from the age of 14 and was hospitalized in 1980, at the age of 25 years, because of hypertension and proteinuria (1.5 g/day). Thrombocytopenia was found with an abundance of megakaryocytes in the bone marrow. Both steroid treatment and splenectomy failed to ameliorate the thrombocytopenia, thought to be due to idiopathic thrombocytopenic purpura. Progressive renal failure, secondary hyperparathyroidism and uremic osteodystrophy were diagnosed in 1995. In January 1996, when she was hospitalized because of high-grade fever, we saw giant platelets and prominent blue inclusion bodies in almost all granulocytes in the peripheral blood smear. Electron microscopy confirmed the diagnosis of May-Hegglin anomaly in this patient and one of her sons, who at that time showed thrombocytopenia but no renal disease. Three years later, however, at the age of 15, the affected son was found to develop proteinuria. Coexpression of the May-Hegglin anomaly and renal disease, reported previously in a few other patients, may in fact represent a new subentity.

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 97%

Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

Demeter

Lelkes

Nemes

et al. 2001

Annals of Hematology

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“…Nevertheless, occasionally bleeding time if prolonged may endanger organs or even life [14]. One intracerebral hemorrhage [15] and other fatal hemorrhages in affected relatives with this disorder have been reported [16,17].…”

Section: Discussionmentioning

confidence: 99%

“…In our patient's family were three members known to have MHA who underwent splenectomy but did not show any improvement in their platelet count or any decrease in their bleeding tendencies. In the literature, a case has been described where an 11-year-old girl with MHA and severe thrombocytopenia presented with sudden visual loss from intraocular hemorrhage underwent emergent splenectomy and showed some partial response in terms of stabilization of her platelet counts [14]. Subsequently, she had bilateral vitrectomy performed with improvement in her visual acuity.…”

Section: Discussionmentioning

confidence: 99%

Perioperative management of a patient with May-Hegglin anomaly requiring craniotomy

2005

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May-Hegglin anomaly (MHA) is a rare type of autosomal dominant platelet disorder associated with mutations in the gene encoding nonmuscle myosin heavy chain 9 (MYH9). It is characterized by the presence of large platelets, leukocyte inclusions, and thrombocytopenia. The bleeding tendency is usually mild, but severe hemorrhages have been reported. This is the first reported case of a patient with MHA who underwent craniotomy for intractable seizure disorder of temporal lobe origin. Patients who have thrombocytopenia have a higher likelihood of developing intraoperative or postoperative intracranial hematoma and bleeding complications. The patient was administered desmopressin (DDAVP) prior to the neurosurgical procedure and had no complications. With this approach, the use of platelet concentrates could be avoided. We discuss the role of DDAVP in MHA and related platelet disorders and review the current literature. Am.

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“…No abnormality could be detected in either the membranes [ 3 ] or the B-globulin content [4] of these platelets. Although severe bleeding diathesis due to thrombocytopenia or increased bleeding time happens rarely, it appears that, in the event of this occurring with threat to life or organs (e.g., intraocular hemorrhage), splenectomy may be considered, especially if splenic sequestration can be demonstrated [ 5 ] . 0 1992 Wiley-Liss, Inc.…”

Section: Introductionmentioning

confidence: 99%

Coincidental finding of May‐Hegglin anomaly in a patient with end‐stage renal failure

et al. 1992

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We present two cases of May-Hegglin anomaly incidentally discovered in a patient and his brother during investigation of the patient for end-stage renal failure and workup for renal transplantation. Routine laboratory tests were performed and included a basically normal clotting profile. Ultrastructural studies of the May-Hegglin inclusions proved diagnostic, findings were compared with those of two similar granulocyte inclusion bodies, and nomenclature discrepancies that still exist in most references are again emphasized. The finding of the May-Hegglin anomaly in our patient appears to be incidental to the underlying renal disease. A successful renal transplant has been carried out in this patient. We now report on a patient and his brother in which the MHA was discovered during workup of the patient for end-stage renal failure and renal transplantation. No association between the underlying renal disease and the MHA could be demonstrated.

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Successful Splenectomy in May-Hegglin Anomaly: Report of a Case with Platelet Kinetic Studies

Cited by 5 publications

References 2 publications

Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

Familial occurrence of the May-Hegglin anomaly: is the accompanying renal failure part of a new subentity?

Perioperative management of a patient with May-Hegglin anomaly requiring craniotomy

Coincidental finding of May‐Hegglin anomaly in a patient with end‐stage renal failure

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