Skip to main content
main-content

Tipp

Weitere Artikel dieser Ausgabe durch Wischen aufrufen

Erschienen in: Wiener klinische Wochenschrift 17-18/2015

01.09.2015 | Clinical-Pathological Conference

Clinical-Pathological Conference Series from the Medical University of Graz Case No 155: 26-year-old woman in third trimester of pregnancy with epigastric pain and thrombocytopenia

verfasst von: PhD Elisabeth Fabian, MD Florian Eisner, MD Ingrid Pabinger, MD Christian Viertler, MD Sigrid Regauer, MD Andreas Lueger, MD Peter Neumeister, MD Eva-Christine Weiss, MD Wolfgang Schöll, MD Uwe Lang, MD Florian Prüller, MD, AGAF Guenter J. Krejs

Erschienen in: Wiener klinische Wochenschrift | Ausgabe 17-18/2015

Einloggen, um Zugang zu erhalten
share
TEILEN

Excerpt

Due to epigastric pain, the unemployed pregnant woman had been examined at the outpatient clinic of the Styrian Public Health Service 2 months earlier. At that time, all laboratory tests were reportedly within normal limits and the epigastric pain was said not to be unusual during pregnancy. However, when the symptoms became more severe, she presented at the Obstetrics Outpatient Clinic at the University Medical Center in Graz, where thrombocytopenia with a platelet count of 9 G/l (normal: 140–440 G/l) was diagnosed. Other laboratory test results were: leukocytes 15.6 G/l (normal: 4.4–11.3 G/l), erythrocytes 2.4 T/l (normal: 4.1–5.1 T/l), hemoglobin (Hb) 7.8 g/dl (normal: 12.0–15.3 g/dl), hematocrit (Htc) 20.9 % (normal: 35–45 %), mean corpuscular volume (MCV) 86.7 fl (normal: 80–98 fl); differential blood count: 78 % neutrophils (normal: 50–75 %), 13 % lymphocytes (normal: 20–40 %); 100 ‰ reticulocytes (normal: 5–20 ‰), haptoglobin < 0.08 g/l (normal: 0.3–2.0 g/l), Hb in plasma 11.7 mg/dl (normal: < 5 mg/dl), total bilirubin 1.45 mg/dl (normal: 0.1–1.2 mg/dl), alkaline phosphatase (AP) 146 U/l (normal: 35–105 U/l), gamma-glutamyl-transpeptidase (GGT) 22 U/l (normal: < 38 U/l), aspartate-aminotransferase (AST) 86 U/l (normal: < 30 U/l), alanine-aminotransferase (ALT) 15 U/l (normal: < 35 U/l), creatinine 1.25 mg/dl (normal: < 1.0 mg/dl), urea 55 mg/dl (normal: 10–45 mg/dl), uric acid 7.8 mg/dl (normal: 2.4–5.7 mg/dl), glomerular filtration rate (GFR) 59 ml/min/l (normal: 80–140 ml/min/l), creatine kinase (CK) 249 U/l (normal: < 145 U/l), lactate dehydrogenase (LDH) 1719 U/l (normal: 120–240 U/l), glucose 92 mg/dl (normal: 70–115 mg/dl), prothrombin time (PT) 100 %, PT-INR 1.01, activated partial thromboplastin time (APTT) 28.5 s (normal: 26.0–36.0 s), fibrinogen 409 mg/dl (normal: 210–400 mg/dl), antithrombin (AT) 62 % (normal: > 75 %); C-reactive protein (CRP) 17.4 mg/l (normal: < 5 mg/l); urine test tape: Hb markedly increased, urinary protein 1377 mg/l (normal: < 130 mg/l), and 2025 mg/g creatinine (normal: < 110 mg/g creatinine). Since HELLP ( hemolysis, elevated liver enzymes, low platelet count) syndrome was the suspected diagnosis for this primipara in her 36th week of pregnancy, after infusion of two platelet concentrates and one unit of packed red cells, a cesarian section was performed and a healthy boy delivered. There were no complications and afterwards the platelet count was 48 G/l, but there was no further improvement of her thrombocytopenia. Four days post partum laboratory tests showed: platelets 23 G/l, leukocytes 15.2 G/l, erythrocytes 2.6 T/l, Hb 8.1 g/dl, Htc 22.5 %, MCV 85.9 fl, reticulocytes 113 ‰, creatinine 1.15 mg/dl, urea 61 mg/dl, uric acid 8.9 mg/dl, GFR 65.4 ml/min/l, total bilirubin 1.8 mg/dl, AP 96 U/l, GGT 21 U/l, AST 83 U/l, ALT 21 U/l, CK 361 U/l, LDH 2331 U/L, and CRP 23.6 mg/l; fragmented red blood cells were seen on a blood smear. Plasmapheresis was planned and the patient was transferred to the ICU. When serum creatinine increased to 4 mg/dl, hemodialysis was ordered. Serum LDH increased to 3133 U/l; several immunopathological parameters (antinuclear antibodies (ANA), antineutrophil cytoplasmic auto-antibodies against myeloperoxidase (ANCA-MPO) and proteinase 3 (ANCA-Pr3), antibodies against double-strand DNA, mitochondria, smooth muscle cells, and basal membrane) and blood cultures were negative. Daily plasmapheresis (for 8 days) did not raise the platelet count. The patient was increasingly agitated and difficult to calm down. Computer tomography of the head was unremarkable. The patient developed progressive cardiorespiratory instability (troponin-T 2530 pg/ml, normal < 14 pg/ml) and suffered repeated apnea. …
Literatur
1.
Zurück zum Zitat Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socié G, International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106(12):3699–709. PubMedCentralCrossRefPubMed Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socié G, International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood. 2005;106(12):3699–709. PubMedCentralCrossRefPubMed
2.
Zurück zum Zitat Nester CM, Thomas CP. Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated? Hematol Am Soc Hematol Educ Program. 2012;2012:617–25. Nester CM, Thomas CP. Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated? Hematol Am Soc Hematol Educ Program. 2012;2012:617–25.
3.
Zurück zum Zitat Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol. 1996;175(2):460–4. CrossRefPubMed Audibert F, Friedman SA, Frangieh AY, Sibai BM. Clinical utility of strict diagnostic criteria for the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. Am J Obstet Gynecol. 1996;175(2):460–4. CrossRefPubMed
4.
Zurück zum Zitat Martin JN Jr., Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006;195(4):914–34. CrossRefPubMed Martin JN Jr., Rose CH, Briery CM. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006;195(4):914–34. CrossRefPubMed
5.
Zurück zum Zitat Steegers EA, von Dadelszen P, Duvekat JJ, Pijnenborg R. Pre-eclampsia. Lancet. 2010;376(9741):631–44. CrossRefPubMed Steegers EA, von Dadelszen P, Duvekat JJ, Pijnenborg R. Pre-eclampsia. Lancet. 2010;376(9741):631–44. CrossRefPubMed
6.
Zurück zum Zitat Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, How H, Sibai B. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol. 2009;200(4):381.e1–6. CrossRef Stella CL, Dacus J, Guzman E, Dhillon P, Coppage K, How H, Sibai B. The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals. Am J Obstet Gynecol. 2009;200(4):381.e1–6. CrossRef
7.
Zurück zum Zitat Kappler S, Ronan-Bentle S, Graham A. Thrombotic microangiopathies (TTP, HUS, HELLP). Emerg Med Clin North Am. 2014;32(3):649–71. CrossRefPubMed Kappler S, Ronan-Bentle S, Graham A. Thrombotic microangiopathies (TTP, HUS, HELLP). Emerg Med Clin North Am. 2014;32(3):649–71. CrossRefPubMed
8.
Zurück zum Zitat Clark WF. Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial. 2012;25(2):214–9. CrossRefPubMed Clark WF. Thrombotic microangiopathy: current knowledge and outcomes with plasma exchange. Semin Dial. 2012;25(2):214–9. CrossRefPubMed
9.
Zurück zum Zitat Tsai HM. Autoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management. Semin Thromb Hemost. 2012;38(5):469–82. CrossRefPubMed Tsai HM. Autoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management. Semin Thromb Hemost. 2012;38(5):469–82. CrossRefPubMed
10.
Zurück zum Zitat George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66. CrossRefPubMed George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654–66. CrossRefPubMed
11.
Zurück zum Zitat Zhou W, Inada M, Lee TP, Benten D, Lyubsky S, Bouhassira EE, Gupta S, Tsai HM. ADAMTS13 is expressed in hepatic stellate cells. Lab Invest. 2005;85(6):780–8. PubMedCentralCrossRefPubMed Zhou W, Inada M, Lee TP, Benten D, Lyubsky S, Bouhassira EE, Gupta S, Tsai HM. ADAMTS13 is expressed in hepatic stellate cells. Lab Invest. 2005;85(6):780–8. PubMedCentralCrossRefPubMed
12.
Zurück zum Zitat Uemura M, Tatsumi K, Matsumoto M, Fujimoto M, Matsuyama T, Ishikawa M, Iwamoto TA, Mori T, Wanaka A, Fukui H, Fujimura Y. Localization of ADAMTS13 to the stellate cells of human liver. Blood. 2005;106(3):922–4. CrossRefPubMed Uemura M, Tatsumi K, Matsumoto M, Fujimoto M, Matsuyama T, Ishikawa M, Iwamoto TA, Mori T, Wanaka A, Fukui H, Fujimura Y. Localization of ADAMTS13 to the stellate cells of human liver. Blood. 2005;106(3):922–4. CrossRefPubMed
13.
Zurück zum Zitat Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339(22):1585–94. PubMedCentralCrossRefPubMed Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339(22):1585–94. PubMedCentralCrossRefPubMed
14.
Zurück zum Zitat Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H, Bouhassira EE, Upshaw JD Jr., Ginsburg D, Tsai HM. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413(6855):488–94. CrossRefPubMed Levy GG, Nichols WC, Lian EC, Foroud T, McClintick JN, McGee BM, Yang AY, Siemieniak DR, Stark KR, Gruppo R, Sarode R, Shurin SB, Chandrasekaran V, Stabler SP, Sabio H, Bouhassira EE, Upshaw JD Jr., Ginsburg D, Tsai HM. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413(6855):488–94. CrossRefPubMed
15.
Zurück zum Zitat Ferrari S, Mudde GC, Rieger M, Veyradier A, Kremer Hovinga JA, Scheiflinger F. IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2009;7(10):1703–10. CrossRefPubMed Ferrari S, Mudde GC, Rieger M, Veyradier A, Kremer Hovinga JA, Scheiflinger F. IgG subclass distribution of anti-ADAMTS13 antibodies in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2009;7(10):1703–10. CrossRefPubMed
16.
Zurück zum Zitat Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. Proc NY Pathol Soc. 1924;24:21–4. Moschcowitz E. An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. Proc NY Pathol Soc. 1924;24:21–4.
17.
Zurück zum Zitat Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr. 1955;85(38–39):905–9. PubMed Gasser C, Gautier E, Steck A, Siebenmann RE, Oechslin R. Hemolytic-uremic syndrome: bilateral necrosis of the renal cortex in acute acquired hemolytic anemia. Schweiz Med Wochenschr. 1955;85(38–39):905–9. PubMed
18.
Zurück zum Zitat Karmali MA, Petric M, Lim C, Fleming PC, Arbus GS, Lior H. The association between idiopathic hemolytic uremic syndrome and infection by verotoxin-producing Escherichia coli. J Infect Dis. 1985;151(5):775–82. CrossRefPubMed Karmali MA, Petric M, Lim C, Fleming PC, Arbus GS, Lior H. The association between idiopathic hemolytic uremic syndrome and infection by verotoxin-producing Escherichia coli. J Infect Dis. 1985;151(5):775–82. CrossRefPubMed
19.
Zurück zum Zitat Seger R, Joller P, Baerlocher K, Kenny A, Dulake C, Leumann E, Spierig M, Hitzig WH. Hemolytic-uremic syndrome associated with neuraminidase-producing microorganisms: treatment by exchange transfusion. Helv Paediatr Acta. 1980;35(4):359–67. PubMed Seger R, Joller P, Baerlocher K, Kenny A, Dulake C, Leumann E, Spierig M, Hitzig WH. Hemolytic-uremic syndrome associated with neuraminidase-producing microorganisms: treatment by exchange transfusion. Helv Paediatr Acta. 1980;35(4):359–67. PubMed
20.
Zurück zum Zitat Kaplan BS, Chesney RW, Drummond KN. Hemolytic uremic syndrome in families. N Engl J Med. 1975;292(21):1090–3. CrossRefPubMed Kaplan BS, Chesney RW, Drummond KN. Hemolytic uremic syndrome in families. N Engl J Med. 1975;292(21):1090–3. CrossRefPubMed
21.
Zurück zum Zitat Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L, Essig M, Ribes D, Dragon-Durey MA, Bridoux F, Rondeau E, Frémeaux-Bacchi V. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21(5):859–67. PubMedCentralCrossRefPubMed Fakhouri F, Roumenina L, Provot F, Sallée M, Caillard S, Couzi L, Essig M, Ribes D, Dragon-Durey MA, Bridoux F, Rondeau E, Frémeaux-Bacchi V. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21(5):859–67. PubMedCentralCrossRefPubMed
22.
Zurück zum Zitat Ullrich S, Bremer P, Neumann-Grutzeck C, Otto H, Rüther C, von Seydewitz CU, Meyer GP, Ahmadi-Simab K, Röther J, Hogan B, Schwenk W, Fischbach R, Caselitz J, Puttfarcken J, Huggett S, Tiedeken P, Pober J, Kirkiles-Smith NC, Hagenmüller F. Symptoms and clinical course of EHEC O104 infection in hospitalized patients: a prospective single center study. PLoS One. 2013;8(2):e55278. PubMedCentralCrossRefPubMed Ullrich S, Bremer P, Neumann-Grutzeck C, Otto H, Rüther C, von Seydewitz CU, Meyer GP, Ahmadi-Simab K, Röther J, Hogan B, Schwenk W, Fischbach R, Caselitz J, Puttfarcken J, Huggett S, Tiedeken P, Pober J, Kirkiles-Smith NC, Hagenmüller F. Symptoms and clinical course of EHEC O104 infection in hospitalized patients: a prospective single center study. PLoS One. 2013;8(2):e55278. PubMedCentralCrossRefPubMed
23.
Zurück zum Zitat Scully M. Trends in the diagnosis and management of TTP: European perspective. Transfus Apher Sci. 2014:51(1):11–4. CrossRefPubMed Scully M. Trends in the diagnosis and management of TTP: European perspective. Transfus Apher Sci. 2014:51(1):11–4. CrossRefPubMed
24.
Zurück zum Zitat Noris M, Mescia F, Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol. 2012;8(11):622–33. CrossRefPubMed Noris M, Mescia F, Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol. 2012;8(11):622–33. CrossRefPubMed
25.
Zurück zum Zitat Chapin J, Weksler B, Magro C, Laurence J. Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. Br J Haematol. 2012;157(6):772–4. CrossRefPubMed Chapin J, Weksler B, Magro C, Laurence J. Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. Br J Haematol. 2012;157(6):772–4. CrossRefPubMed
26.
Zurück zum Zitat Just S. Methodologies and clinical utility of ADAMTS-13 activity testing. Semin Thromb Hemost. 2010;36(1):82–90. CrossRefPubMed Just S. Methodologies and clinical utility of ADAMTS-13 activity testing. Semin Thromb Hemost. 2010;36(1):82–90. CrossRefPubMed
27.
Zurück zum Zitat Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ, British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323–35. CrossRefPubMed Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Cheung B, Machin SJ, British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158(3):323–35. CrossRefPubMed
Metadaten
Titel
Clinical-Pathological Conference Series from the Medical University of Graz Case No 155: 26-year-old woman in third trimester of pregnancy with epigastric pain and thrombocytopenia
verfasst von
PhD Elisabeth Fabian
MD Florian Eisner
MD Ingrid Pabinger
MD Christian Viertler
MD Sigrid Regauer
MD Andreas Lueger
MD Peter Neumeister
MD Eva-Christine Weiss
MD Wolfgang Schöll
MD Uwe Lang
MD Florian Prüller
MD, AGAF Guenter J. Krejs
Publikationsdatum
01.09.2015
Verlag
Springer Vienna
Erschienen in
Wiener klinische Wochenschrift / Ausgabe 17-18/2015
Print ISSN: 0043-5325
Elektronische ISSN: 1613-7671
DOI
https://doi.org/10.1007/s00508-015-0829-0

Weitere Artikel der Ausgabe 17-18/2015

Wiener klinische Wochenschrift 17-18/2015 Zur Ausgabe

mitteilungen der gesellschaft der ärzte in wien

Veranstaltungen

mitteilungen der gesellschaft

ÖGIM FLIP