History

Fact Explanation
Introduction Microangiopathic Autoimmune Hemolytic anemia is a thrombotic microangiopathy, that occurred due to the endothelial injury of the vessels followed by the activation of clotting system inside the microvasculature forming microthrombi. [12] When the red blood cells are passing through these micrthrombi, they will undergo destruction causing haemolyss. MAHA is associated with various disease conditions including idiopathic thrombotic thrombocytopenic purpura (TTP), [12] disseminated intravascular coagulation (DIC), hemolytic uremic syndrome (HUS), and vasculitis. This may be familial, idiopathic or acquired secondary to toxins, pregnancy, infections (e.g., HIV, certain Shigella and E. coli) and drugs. [12] Patients with some solid organ cancers may also present MAHA. [4] There can be platelet aggregation and thrombus formation, platelet consumption, impaired microvascular blood flow, and mechanical destruction of erythrocytes. [5]
Lethargy, Malaise, fatigue Anaemia causes reduced blood oxygenation, leading to reduced supply of oxygen to the energy production. Therefore they feel lack of energy. [2]
Dark urine Suggestive of intravascular haemolysis leading to haemoglobinuria. [1]
Right hypochondrial pain May be present due to associated gallstones due to high level of bilirubin in the blood. [3]
Features of thrombotic thrombocytopenic purpura: fever, visual disturbances, mental state changes, urinary problems Thrombotic thrombocytopenic purpura (TTP) is a disease where MAHA is associated with the other features due to the vascular occlusions of the microcirculation. [7]
Features of Haemolytic uraemic syndrome: diarrhoea, abdominal pain, seizures etc [8, 11] Shiga toxin–producing Escherichia coli are thecause for the diarrhea-associated HUS. [9] It causes haemolysis with mocroangiopathic changes. [8]
Factors in favour of of Disseminated intravascular Coagulation Disseminated intravascular coagulation (DIC) occurs due to sequential activation of the coagulation and fibrinolytic system. Trauma and sepsis are the most common precipitating factors. [6]
History of malignancy Microangiopathic hemolytic anemia (MAHA) occurs occasionally as a paraneoplastic syndrome in association with some solid tumors, such as gastric, breast, and lung cancers. [4] MAHA occurs late in the course of disease, in malignant infiltration of bone marrow, or as a side-effect of chemotherapeutic agents. [5]
Bone pain and respiratory symptoms These symptoms are observed in patients with cancer associated MAHA. [2]
Pregnancy HELLP syndrome may be a cause of MAHA in pregnant mothers. [12] Severe preeclampsis is associated with HEELP syndrome [10] with haemolysis, elevated and low platelets.
History of infections HIV is a known association with the MAHA. [12]
History of drug use Common drugs causing MAHA includes quinine, cyclosporine, ticlopidine, clopidogrel. [12]
References
  1. GUPTA S, SZERSZEN A, NAKHL F, VARMA S, GOTTESMAN A, FORTE F, DHAR M. Severe refractory autoimmune hemolytic anemia with both warm and cold autoantibodies that responded completely to a single cycle of rituximab: a case report J Med Case Reports [online] :156 [viewed 08 August 2014] Available from: doi:10.1186/1752-1947-5-156
  2. NALESNIK JG, MYSLIWIEC AG, CANBY-HAGINO E. Anemia in Men with Advanced Prostate Cancer: Incidence, Etiology, and Treatment Rev Urol [online] 2004, 6(1):1-4 [viewed 18 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1472681
  3. MCSHERRY CK, FERSTENBERG H, CALHOUN WF, LAHMAN E, VIRSHUP M. The natural history of diagnosed gallstone disease in symptomatic and asymptomatic patients. Ann Surg [online] 1985 Jul, 202(1):59-63 [viewed 18 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1250837
  4. SHIN SY, PARK H, CHAE SW, WOO HY. Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature Korean J Lab Med [online] 2011 Jul, 31(3):157-161 [viewed 20 November 2014] Available from: doi:10.3343/kjlm.2011.31.3.157
  5. BUTLER MJ, YIN M, QUDDUS F. Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy Hematol Rep [online] , 6(1):5159 [viewed 20 November 2014] Available from: doi:10.4081/hr.2014.5159
  6. SOMASHEKHAR M, KADAMBA PS, WAKODKAR M. Chronic disseminated intravascular coagulation presenting as renal mass J Indian Assoc Pediatr Surg [online] 2008, 13(4):144-146 [viewed 20 November 2014] Available from: doi:10.4103/0971-9261.44767
  7. RUTKOW IM. Thrombotic thrombocytopenic purpura (TTP) and splenectomy: a current appraisal. Ann Surg [online] 1978 Nov, 188(5):701-705 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1396769
  8. TSERENPUNTSAG B, CHANG HG, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Risk and Escherichia coli O157:H7 Emerg Infect Dis [online] 2005 Dec, 11(12):1955-1957 [viewed 20 November 2014] Available from: doi:10.3201/eid1112.050607
  9. CHANG HG, TSERENPUNTSAG B, KACICA M, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Incidence in New York Emerg Infect Dis [online] 2004 May, 10(5):928-931 [viewed 20 November 2014] Available from: doi:10.3201/eid1005.030456
  10. HARAM KJELL, MORTENSEN JAN HELGE, NAGY BáLINT. Genetic Aspects of Preeclampsia and the HELLP Syndrome. Journal of Pregnancy [online] 2014 December, 2014:1-13 [viewed 20 November 2014] Available from: doi:10.1155/2014/910751
  11. NATHANSON S, KWON T, ELMALEH M, CHARBIT M, LAUNAY EA, HARAMBAT J, BRUN M, RANCHIN B, BANDIN F, CLOAREC S, BOURDAT-MICHEL G, PIèTREMENT C, CHAMPION G, ULINSKI T, DESCHêNES G. Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome Clin J Am Soc Nephrol [online] 2010 Jul, 5(7):1218-1228 [viewed 20 November 2014] Available from: doi:10.2215/CJN.08921209
  12. MINTZER DAVID M., BILLET SHIRA N., CHMIELEWSKI LAUREN. Drug-Induced Hematologic Syndromes. Advances in Hematology [online] 2009 December, 2009:1-11 [viewed 20 November 2014] Available from: doi:10.1155/2009/495863

Examination

Fact Explanation
Pallor Haemoglobin level reduces during an attack, patient develops anaemia due to red cell destruction. [4]
Icterus Haemolysis of red blood cells releases bilirubin into the blood causing elevation of unconjugated bilirubin. This accumulated bilirubin causes yellowish discolouration of eyes and mucous membranes. [5]
Wasting Solid organ malignancies are known to be associated with the MAHA as a paraneoplastic syndrome. [4]
Right hypochondrial tendreness Associated with gallstones due to high level of bilirubin in the blood. [2]
Dyspnea It can be either as a result of anemia [5] Respiratory distress is seen in associated lung carcinoma, as MAHA occurs as a paraneoplstic syndrome in such malignancies. [4]
Breast lump, abdominal lump, features of lung mass (reduced chest expansion, dull percussion note,increased vocal fremitus, reduced breath sounds) Microangiopathic hemolytic anemia (MAHA) occurs occasionally as a paraneoplastic syndrome in association with some solid tumors, such as gastric, breast, and lung cancers. [4]
Focal neurological signs Brain parenchymal ischemia or hemorrhages may occur due to the thrombotic occlusion of either carotids or vertebral arteries. [3]
Fundal examination There will be haemorrhages seen on funduscopy. [1]
References
  1. FINKELSTEIN R, MARKEL A, CARTER A, BROOK JG. Plasma infusions in thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus--a successful outcome Postgrad Med J [online] 1982 Sep, 58(683):577-579 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2426448
  2. MCSHERRY CK, FERSTENBERG H, CALHOUN WF, LAHMAN E, VIRSHUP M. The natural history of diagnosed gallstone disease in symptomatic and asymptomatic patients. Ann Surg [online] 1985 Jul, 202(1):59-63 [viewed 18 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1250837
  3. NATHANSON S, KWON T, ELMALEH M, CHARBIT M, LAUNAY EA, HARAMBAT J, BRUN M, RANCHIN B, BANDIN F, CLOAREC S, BOURDAT-MICHEL G, PIèTREMENT C, CHAMPION G, ULINSKI T, DESCHêNES G. Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome Clin J Am Soc Nephrol [online] 2010 Jul, 5(7):1218-1228 [viewed 20 November 2014] Available from: doi:10.2215/CJN.08921209
  4. SHIN SY, PARK H, CHAE SW, WOO HY. Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature Korean J Lab Med [online] 2011 Jul, 31(3):157-161 [viewed 20 November 2014] Available from: doi:10.3343/kjlm.2011.31.3.157
  5. DHALIWAL G, CORNETT PA, TIERNEY LM JR. Hemolytic anemia. Am Fam Physician [online] 2004 Jun 1, 69(11):2599-606 [viewed 01 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/15202694

Differential Diagnoses

Fact Explanation
Associated conditions: Thrombotic thrombocytopenic purpura Thrombotic thrombocytopenic purpura (TTP) is a disease associated with microangiopathic anemia, fever, neurologic manifestations, renal abnormalities, and thrombocytopenia. These abnormalities are due to the vascular occlusions of the microcirculation. Treatment is usually splenectomy. [10]
Associated conditions: Haemolytic uraemic syndrome HUS is associated with acute diarrheain combination with hemolytic anemia with microangiopathic changes, renal insufficiency. [11] Pathogenesis involves platelet aggregation and thrombus formation, resulting in platelet consumption, impaired microvascular blood flow, and mechanical destruction of erythrocytes. [8] Creatinine levelwill be elevated and thrombocytopenia (platelet count <150,000/μL) will be present. [11]
Associated conditions: Cancer associated Microangiopathic Haemolytic Anaemia MAHA is an occasional complication of solid malignancies,particularly in association with gastric, breast, prostate, and lung cancers. [9] It occurs late in the course of disease, in malignant infiltration of bone marrow. Cancer-associated MAHA can be differentiated from classic TTP, as MAHA may have normal or variable levels of ADAMTS13 activity, and is not responding to therapeutic plasma exchange. [8] There can be other clinical findings that would be helpful in arriving at a diagnosis in relation to the cancer. CT scan, ultrasoundscan and mammogram are useful investigations for screening for underlying malignancies. [9]
Hereditory spherocytosis [5] This is an autosomal dominant red blood cell membrane defect with spherical red blood cells undergo extravascular haemolysis. There will be spherocytes in the blood film and increased osmotic fragility apart from the other findings of haemolytic anaemia. [5]
Glucose 6 phosphate dehydrogenase deficiency This is an X-linked condition [3] where attacks are precipitated by the reduced glutathione production in oxidative stresses as in ingestion of fava beans, drugs like as primaquine, sulfonamide and aspirin or illness. Blister cells and bite cells are seen on blood film with reduction of enzyme level after 8 weeks of attack. [3]
Autoimmune haemolytic anaemia There are 2 types of autoimmune haemolytic anaemia: warm and cold. Warm type is IgG mediated at temperature less than 4 centigrades and cold type is IgM mediated at temperature 37 centigrades. [6] This causes extra vascular haemolysis. Direct and indirect coomb’s test are positive [2] and there will be spherocytes in the blood film. [1]
Paroxysmal nocturnal haemoglobonuria This arises due to the acquired somatic mutation in the X-linked phosphatidylinositol glycan class A gene. Chronic intravascular hemolysis, occur with resulting in hemolytic anemia and hemosiderinuria. Bone marrow failure and thromboembolia are the other comlications of PNH. There is mild to severe anemia with moderate reticulocytosis and mild jaundice, with negative Coombs test. Dark urine and urinary hemosiderin, are suggestive of intravascular hemolysis. [7]
References
  1. NANDENNAVAR M, CYRIAC S, KRISHNAKUMAR, SAGAR T. Immune Hemolytic Anemia in a Patient with Tuberculous Lymphadenitis J Glob Infect Dis [online] 2011, 3(1):89-91 [viewed 22 September 2014] Available from: doi:10.4103/0974-777X.77303
  2. ALWAR V, SHANTHALA DA, SITALAKSHMI S, KARUNA RK. Clinical Patterns and Hematological Spectrum in Autoimmune Hemolytic Anemia J Lab Physicians [online] 2010, 2(1):17-20 [viewed 22 September 2014] Available from: doi:10.4103/0974-2727.66703
  3. PETERS AL, VAN NOORDEN CJ. Glucose-6-phosphate Dehydrogenase Deficiency and Malaria: Cytochemical Detection of Heterozygous G6PD Deficiency in Women J Histochem Cytochem [online] 2009 Nov, 57(11):1003-1011 [viewed 06 August 2014] Available from: doi:10.1369/jhc.2009.953828
  4. VON DEM BORNE AE, ENGELFRIET CP, BECKERS D, VAN DER KORT-HENKES G, VAN DER GIESSEN M, VAN LOGHEM JJ. Autoimmune haemolytic anaemias. II. Warm haemolysins--serological and immunochemical investigations and 51Cr studies Clin Exp Immunol [online] 1969 Mar, 4(3):333-343 [viewed 18 September 2014] Available from:
  5. DAS A, BANSAL D, DAS R, TREHAN A, MARWAHA RK. Hereditary spherocytosis in children: profile and post-splenectomy outcome. Indian Pediatr [online] 2014 Feb, 51(2):139-41 [viewed 12 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/24632695
  6. PALLA AR, KHIMANI F, CRAIG MD. Warm Autoimmune Hemolytic Anemia with a Direct Antiglobulin Test Positive for C3 and Negative for IgG: A Case Study and Analytical Literature Review of Incidence and Severity Clin Med Insights Case Rep [online] :57-60 [viewed 22 September 2014] Available from: doi:10.4137/CCRep.S11469
  7. WILEY JS. Red cell survival studies in hereditary spherocytosis J Clin INVEST [online] 1970 Apr, 49(4):666-672 [viewed 22 September 2014] Available from: doi:10.1172/JCI106278
  8. BUTLER MJ, YIN M, QUDDUS F. Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy Hematol Rep [online] , 6(1):5159 [viewed 20 November 2014] Available from: doi:10.4081/hr.2014.5159
  9. SHIN SY, PARK H, CHAE SW, WOO HY. Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature Korean J Lab Med [online] 2011 Jul, 31(3):157-161 [viewed 20 November 2014] Available from: doi:10.3343/kjlm.2011.31.3.157
  10. RUTKOW IM. Thrombotic thrombocytopenic purpura (TTP) and splenectomy: a current appraisal. Ann Surg [online] 1978 Nov, 188(5):701-705 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1396769
  11. TSERENPUNTSAG B, CHANG HG, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Risk and Escherichia coli O157:H7 Emerg Infect Dis [online] 2005 Dec, 11(12):1955-1957 [viewed 20 November 2014] Available from: doi:10.3201/eid1112.050607

Investigations - for Diagnosis

Fact Explanation
Full blood count With low level of Hemoglobin levels due to anemia, reticulocytosis will be evident as a result of hemolysis. [5]. Blood film may show thrombocytopenia in cancer associated MAHA [1] , disseminated intravascular coagulation [3] and many other diseases associated with MAHA. Platelet count may also be reduced in HELLP syndrome. [4]
Blood picture This help to distinguish the other forms of anemia [5] Schistocytes are seen on the blood film due to RBC fragmentation. Polychromasia, nucleated red blood cells are also seen on the blood smear. [1] Leucoberythrobastic picture will be seen particularly in malignancy associated MAHA. [2]
Haptoglobin level Haptoglobin is involved with binding free Hemoglobin in the blood. Decreased haptoglobin levels are seen in association with intravascular haemolysis. [1]
Direct Coombs' test Negative direct Coombs' test [1] is helpful in excludin autoimmune conditions.
Serum levels of total bilirubin Elevated serum levels of total bilirubin is seen due to haemolysis. [1]
Lactate dehydrogenase (LDH) LDH levels are increased due to increased cell breakdown. [1]
Prothrombin time Prolonged prothrombin time is seen in disseminated intravascular coagulation. [3]
References
  1. SHIN SY, PARK H, CHAE SW, WOO HY. Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature Korean J Lab Med [online] 2011 Jul, 31(3):157-161 [viewed 20 November 2014] Available from: doi:10.3343/kjlm.2011.31.3.157
  2. BUTLER MJ, YIN M, QUDDUS F. Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy Hematol Rep [online] , 6(1):5159 [viewed 20 November 2014] Available from: doi:10.4081/hr.2014.5159
  3. WILDE JT, ROBERTS KM, GREAVES M, PRESTON FE. Association between necropsy evidence of disseminated intravascular coagulation and coagulation variables before death in patients in intensive care units. J Clin Pathol [online] 1988 Feb, 41(2):138-142 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1141367
  4. SERENPUNTSAG B, CHANG HG, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Risk and Escherichia coli O157:H7 Emerg Infect Dis [online] 2005 Dec, 11(12):1955-1957 [viewed 20 November 2014] Available from: doi:10.3201/eid1112.05060
  5. DHALIWAL G, CORNETT PA, TIERNEY LM JR. Hemolytic anemia. Am Fam Physician [online] 2004 Jun 1, 69(11):2599-606 [viewed 01 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/152026

Investigations - Fitness for Management

Fact Explanation
Urinalysis [1] Urinalysis may be needed in the cases where they present with dark urine to see any red blood cells present in the urine. [1]
Stool examination/culture Stool of the diarrohoea associated MAHA patients should be cultured for E. coli. [2]
References
  1. SUDHA REDDY VR, SAMAYAM P, RAVICHANDER B, BAI U. Autoimmune Hemolytic Anemia: Mixed Type--A Case Report Indian J Hematol Blood Transfus [online] 2011 Jun, 27(2):107-110 [viewed 08 August 2014] Available from: doi:10.1007/s12288-011-0065-3
  2. TSERENPUNTSAG B, CHANG HG, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Risk and Escherichia coli O157:H7 Emerg Infect Dis [online] 2005 Dec, 11(12):1955-1957 [viewed 20 November 2014] Available from: doi:10.3201/eid1112.050607

Investigations - Followup

Fact Explanation
Renal function tests(Serum creatinine, serum electroytes and blood urea) Diarrhea-associated hemolytic uremic syndrome (HUS) causes acute renal failure particularly in children who are younger than 5 years. [1]
References
  1. NATHANSON S, KWON T, ELMALEH M, CHARBIT M, LAUNAY EA, HARAMBAT J, BRUN M, RANCHIN B, BANDIN F, CLOAREC S, BOURDAT-MICHEL G, PIèTREMENT C, CHAMPION G, ULINSKI T, DESCHêNES G. Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome Clin J Am Soc Nephrol [online] 2010 Jul, 5(7):1218-1228 [viewed 20 November 2014] Available from: doi:10.2215/CJN.08921209

Investigations - Screening/Staging

Fact Explanation
Endoscopy of the upper and lower gastrointestinal tract, abdominal ultrasonograph, thoracic, abdominal, and pelvic computed tomography Solid organ malignancies such as lung, breast and stomach are occasionally a cause for MAHA. Therefore these investigations are done in order to rule out the possibility of solid tumor origin. [1]
Bone marrow examination Signet ring cell carcinoma is a malignancy associated with MAHA. Bone marrow metastases of signet ring cell carcinoma with extensive bone marrow necrosis will be seen in such patients. [1]
D-dimer, fibrinogen and fibrin degradation products (FDP) Important to exclude the disseminated intravascular coagulation DIC) as they will have elevated D dimer levels and reduced fibrinogen. FDP will be elevated due to the fibrin degradation in DIC. [3]
Cold agglutinin assay /Serum IgG and IgM immunoglobulins To rule out the warm and cold types of autoimmune haemolytic anaemia. [2]
References
  1. SHIN SY, PARK H, CHAE SW, WOO HY. Microangiopathic Hemolytic Anemia as the First Manifestation of Metastatic Signet Ring Cell Carcinoma of Unknown Origin: A Case Report and Review of Literature Korean J Lab Med [online] 2011 Jul, 31(3):157-161 [viewed 20 November 2014] Available from: doi:10.3343/kjlm.2011.31.3.157
  2. BUTLER MJ, YIN M, QUDDUS F. Isolated Hemolytic Anemia: An Unusual Manifestation of Occult Malignancy Hematol Rep [online] , 6(1):5159 [viewed 20 November 2014] Available from: doi:10.4081/hr.2014.5159
  3. SOMASHEKHAR M, KADAMBA PS, WAKODKAR M. Chronic disseminated intravascular coagulation presenting as renal mass J Indian Assoc Pediatr Surg [online] 2008, 13(4):144-146 [viewed 20 November 2014] Available from: doi:10.4103/0971-9261.44767

Management - General Measures

Fact Explanation
Patient education Patient should be educated about the nature of the disease, particularly about the underlying disease condition such as thrombotic thrombocytopenic purpura, dissemnated intravascular coagulation [4] and its complications and treatment options.
Supportive treatment Red cell transfusion may be needed in some occasions with severe anemia. [3] Fresh frozen plasma may be required in thrombotic thrombocytopenic purpura to increase the thrombocytes. [2]
Monitoring for complications of the treatment With the long term treatment of microangiopathic haemolytic anaemia, especially with prednisolone they can develop complications due to the treatment such as hypertension, glaucoma, severe osteoporosis, and osteoarthritis. [1]
References
  1. PETERS AL, VAN NOORDEN CJ. Glucose-6-phosphate Dehydrogenase Deficiency and Malaria: Cytochemical Detection of Heterozygous G6PD Deficiency in Women J Histochem Cytochem [online] 2009 Nov, 57(11):1003-1011 [viewed 06 August 2014] Available from: doi:10.1369/jhc.2009.953828
  2. FINKELSTEIN R, MARKEL A, CARTER A, BROOK JG. Plasma infusions in thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus--a successful outcome Postgrad Med J [online] 1982 Sep, 58(683):577-579 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2426448
  3. MAKADIA D, SIDDAIAHGARI SR, LATHA MS. Anti B cell targeted therapy for autoimmune hemolytic anemia in an infant Indian J Pharmacol [online] 2013, 45(5):526-527 [viewed 06 August 2014] Available from: doi:10.4103/0253-7613.117755
  4. MINTZER DAVID M., BILLET SHIRA N., CHMIELEWSKI LAUREN. Drug-Induced Hematologic Syndromes. Advances in Hematology [online] 2009 December, 2009:1-11 [viewed 20 November 2014] Available from: doi:10.1155/2009/495863

Management - Specific Treatments

Fact Explanation
Management options Management will be depend on the underlying disease condition of the patient. [4] Drugs causing MAHA such as quinine, cyclosporine, ticlopidine, clopidogrel [7] may need to be stopped where appropriate. Severe preeclampsia associated HEELP syndrome [8] with haemolysis, elevated and low platelets may require delivery of the fetus.
Management of Thrombotic thrombocytopenic purpura (TTP) Splenectomy is considered as a method of initial management of all patients with TTP. Other management options includes high dose corticosteroids and antiplatelet drugs. [1] Plasma exchange and plasmapharesis to replace the plasma is also an effective treatment for TTP. [4]
Management of Haemolytic uraemic syndrome Due to the low plasma vitamin E concentrations in patients with haemolytic uraemic syndrome, treatment with the antioxidant vitamin E is gaining popular for the treatment of HUS. [3]
Management of disseminated intravascular coagulation Management of DIC is targeted to treat the underlying pathogenesis. Infections, trauma, liver disease are the common underlying causes of DIC. Infections need appropriate treatment with antibiotics. [5] Plasma and platelet transfusion has to be use cautiously toprevent stimulation of the coagulation system. Available options for the treatment of DIC include plasma and platelet transfusion, anticoagulant administration, and coagulation inhibitor administration. [6]
References
  1. RUTKOW IM. Thrombotic thrombocytopenic purpura (TTP) and splenectomy: a current appraisal. Ann Surg [online] 1978 Nov, 188(5):701-705 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1396769
  2. NATHANSON S, KWON T, ELMALEH M, CHARBIT M, LAUNAY EA, HARAMBAT J, BRUN M, RANCHIN B, BANDIN F, CLOAREC S, BOURDAT-MICHEL G, PIèTREMENT C, CHAMPION G, ULINSKI T, DESCHêNES G. Acute Neurological Involvement in Diarrhea-Associated Hemolytic Uremic Syndrome Clin J Am Soc Nephrol [online] 2010 Jul, 5(7):1218-1228 [viewed 20 November 2014] Available from: doi:10.2215/CJN.08921209
  3. POWELL HR, MCCREDIE DA, TAYLOR CM, BURKE JR, WALKER RG. Vitamin E treatment of haemolytic uraemic syndrome. Arch Dis Child [online] 1984 May, 59(5):401-404 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1628506
  4. FINKELSTEIN R, MARKEL A, CARTER A, BROOK JG. Plasma infusions in thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus--a successful outcome Postgrad Med J [online] 1982 Sep, 58(683):577-579 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2426448
  5. BAGLIN T. Disseminated intravascular coagulation: diagnosis and treatment. BMJ [online] 1996 Mar 16, 312(7032):683-687 [viewed 20 November 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2350519
  6. WALIA SS, WALIA HS. Therapeutic interventions in endotoxin-induced disseminated intravascular coagulation J Nat Sci Biol Med [online] 2012, 3(1):108-109 [viewed 20 November 2014] Available from: doi:10.4103/0976-9668.95989
  7. MINTZER DAVID M., BILLET SHIRA N., CHMIELEWSKI LAUREN. Drug-Induced Hematologic Syndromes. Advances in Hematology [online] 2009 December, 2009:1-11 [viewed 20 November 2014] Available from: doi:10.1155/2009/495863
  8. TSERENPUNTSAG B, CHANG HG, SMITH PF, MORSE DL. Hemolytic Uremic Syndrome Risk and Escherichia coli O157:H7 Emerg Infect Dis [online] 2005 Dec, 11(12):1955-1957 [viewed 20 November 2014] Available from: doi:10.3201/eid1112.05060