Familial Pseudohyperkalemia: A Rare Cause of Hyperkalemia

Sugimoto, Toshiro; Kume, Shinji; Osawa, Norihisa; Nakazawa, Jun; Koya, Daisuke; Kashiwagi, Atsunori

doi:10.2169/internalmedicine.44.875

Cited by 16 publications

(7 citation statements)

References 11 publications

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“…Causes of pseudohyperkalemia include an autosomal dominant familial syndrome in which red blood cells exhibit an abnormal potassium leak,9 10 hereditary spherocytosis,11 leucocytosis of chronic inflammation such as in rheumatoid arthritis and infectious mononucleosis,12 13 chronic myeloid or lymphocytic leucaemia,14 15 essential thrombocythaemia16 17 and reactive postoperative thrombocytosis 18…”

Section: Discussionmentioning

confidence: 99%

Postsplenectomy thrombocytosis with pseudohyperkalaemia

Alizadeh

Hadjinicolaou²,

Hadjittofi

et al. 2015

BMJ Case Reports

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SUMMARYA 52-year old man developed hyperkalaemia on the 11th postoperative day following an extensive open retroperitoneal liposarcoma resection that included splenectomy. Despite thorough investigations, no aetiology for the hyperkalaemia was identified and standard empirical treatment was ineffective. On reconsideration, in view of the patient's concurrent thrombocytosis, a pseudofactual or artefactual hyperkalaemia was suspected. This was confirmed by contemporaneous testing of serum and plasma potassium levels, with the latter value lying within the normal range. Treatment for hyperkalaemia was discontinued, thus averting an iatrogenic and potentially dangerous hypokalaemia. This case highlights pseudohyperkalaemia as an often-neglected cause of elevated serum potassium levels and discusses its association with thrombocytosis following splenectomy. BACKGROUND

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Section: Discussionmentioning

confidence: 99%

Postsplenectomy thrombocytosis with pseudohyperkalaemia

Alizadeh

Hadjinicolaou²,

Hadjittofi

et al. 2015

BMJ Case Reports

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“…A subtype of pseudohyperkalemia has been described as familial pseudohyperkalemia. It is an autosomal dominant disorder characterized by an inherent defect in red blood cell membrane potassium channels, which renders them to leak potassium when incubated at low temperatures particularly below 20° centigrade [3]. …”

Section: Discussionmentioning

confidence: 99%

Unexplained hyperkalemia: The tip of the iceberg

Meka

Malik

2012

Am J Case Rep

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SummaryBackground:Hyperkalemia is a potentially life-threatening medical condition; on the other hand pseudohyperkalemia is a benign entity, which should be suspected when serum potassium is elevated without concomitant electrolyte imbalances or remarkable degree of renal dysfunction. Patients seldom have the classical manifestations of hyperkalemia. Failure to recognize this condition causes anxiety among physicians, unnecessary laboratory testing and unwarranted treatments.Case Report:We describe a sixty-year-old woman with persistent hyperkalemia and mean platelet count over a six-month period of 1015×103 cells/cumm. Based on this finding of thrombocytosis an immediate hematological evaluation has detected a myeloproliferative disorder, specifically essential thrombocythemia. Normalizing platelet count was paralleled by resolution of hyperkalemia.Conclusions:Pseudohyperkalemia might be the tip of the iceberg to a major underlying pathological process. Unless a high index of suspicion to diagnose this disorder is maintained it will continue to be remarkably under diagnosed, subjecting patients to numerous unnecessary tests and treatments.

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“…Other improper phlebotomy techniques that can cause pseudohyperkalemia include mislabeling, prolonged use of the tourniquet and fist clenching (which cause release of potassium from the skeletal muscle to the venous blood), excessive suction being applied to the syringe, phlebotomy site being above an intravenous venous line used to infuse potassium containing solution, delayed separation of blood cells from the serum and contamination by inappropriate anticoagulants such as potassium ethylenediaminetetraacetic acid or fluoride. 4,5 Familial pseudohyperkalemia is an uncommon syndrome first described by Stewart et al in 1979. 6 The RBCs of susceptible people are abnormally sensitive to temperature, and at vitro temperatures undergo membrane damage with potassium leakage.…”

Section: Discussionmentioning

confidence: 99%

Postsplenectomy Thrombocytosis and Pseudohyperkalemia in Trauma: A Case Report and Review of Literature

Ahmed

Isaac

2009

Journal of Trauma: Injury, Infection &Amp; Critical Care

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P seudohyperkalemia as a cause of elevated potassium levels is an under recognized condition, which when missed can result in over treatment with potentially lethal consequences. The diagnosis has been associated classically with thrombocytosis in myeloproliferative conditions, and rarely with trauma induced splenectomy. We present a case of pseudohyperkalemia secondary to posttraumatic splenectomy thrombocytosis, in a previously healthy patient. A literature review yielded only two similar reported cases. CASE REPORTM.I., a 24-year-old Egyptian man with no known medical conditions, was involved in a pedestrian accident while walking across a street. He sustained severe head and chest injuries and was brought to our emergency department with a Glasgow Coma Scale score of 4 over 15. On examination, he was found to be unconscious, hypotensive, tachycardic, and desaturating at room air. He was immediately resuscitated with fluids, intubated and ventilated and sent for emergency computed tomography scans, which showed severe cerebral edema, contusion of left lung with a hemothorax and perisplenic collection. After insertion of a chest drain, he was sent for an exploratory laprotomy, which revealed grade IV splenic injury with a retroperitoneal hematoma in his left lumbar region. A splenectomy was performed and the patient was transferred to our intensive care unit for further management.Our patient recovered hemodynamically and his oxygen requirement decreased; but because of poor neurologic recovery, he had to undergo a tracheostomy. He was also vaccinated against both pneumococci and haemophilus influenzae. During his stay with us, he developed an intermittent fever with mild leucocytosis. A septic workup was performed but found to be negative, and the fever was determined to be central in origin. The white blood count (WBC) receded over time; however, by postoperative day 7, his blood work up showed thrombocytosis, with a platelet count of 1634 ϫ 10 9 and hyperkalemia, the serum potassium measuring 5.6 mEq/L. Electrocardiogram showed no cardiac arrhythmias, renal functions were normal, and he did not seem to have any signs of muscular weakness or respiratory insufficiency. His diet and medications were reviewed, but found to be noncontributory to hyperkalemia. We sent a simultaneous blood sample for plasma potassium, which was found to be 3.8 mEq/L. The diagnosis of pseudohyperkalemia secondary to thrombocytosis was confirmed and no potassium lowering therapy was initiated.

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Familial Pseudohyperkalemia: A Rare Cause of Hyperkalemia

Cited by 16 publications

References 11 publications

Postsplenectomy thrombocytosis with pseudohyperkalaemia

Postsplenectomy thrombocytosis with pseudohyperkalaemia

Unexplained hyperkalemia: The tip of the iceberg

Postsplenectomy Thrombocytosis and Pseudohyperkalemia in Trauma: A Case Report and Review of Literature

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