Case report
peer-reviewed
Published: December 01, 2022 (see history)
DOI: 10.7759/cureus.32095
Cite this nonfiction as: Abosheaishaa H, Nassar M, Ghallab M, et al. (December 01, 2022) Pernicious Anemia and Vitamin B12 Deficiency Presenting As Pseudothrombotic Microangiopathy and Developing Secondary Thrombocytopenia After Treatment: A Case Report. Cureus 14(12): e32095. doi:10.7759/cureus.32095
Abstract
Pernicious anemia (PA) is an autoimmune illness secondary to chronic atrophic gastritis starring to vitamin B12 deficiency. Rarely, immoderate patients whitethorn make precocious hematological complications that mimic those of thrombotic thrombocytopenic purpura (TTP). Differentiating these conditions is important due to the fact that they necessitate antithetic management. We contiguous a lawsuit of a 68-year-old antheral who presented with generalized weakness, fatigue, and shortness of breath. This diligent had anemia, thrombocytopenia, and a markedly deficient serum level of vitamin B12. The symptoms initially mimicked those associated with TTP, but the enactment of ADAMTS 13 was normal. A diagnosis of pseudo-TTP has been made owed to vitamin B12 deficiency resulting from PA with reactive thrombocytosis. Ultimately, vitamin B12 deficiency pseudo-TTP should beryllium considered a differential diagnosis for therapy refractory TTP due to the fact that of its antithetic absorption strategies ranging from parenteral Vitamin B12 successful PA patients with highly favorable outcomes to much precocious attraction with little favorable outcomes successful TTP patients.
Introduction
Pernicious anemia (PA) is an autoimmune illness secondary to chronic atrophic gastritis starring to vitamin B12 deficiency [1]. The diagnosis of PA is often confirmed by the beingness of anti-intrinsic origin antibodies, which, contempt their debased sensitivity, are highly circumstantial [2]. During the aboriginal stages of the disease, patients are usually asymptomatic, contributing to an underestimated diagnosis complaint [2]. As the illness progresses, patients make hematological abnormalities and neuropsychiatric symptoms successful much terrible cases [2]. Hematological findings commonly recovered successful peripheral humor smears see megaloblastic anemia, elevated lactate dehydrogenase (LDH), and hyper-segmented neutrophils [3]. A uncommon information whitethorn effect successful processing much terrible hematological complications, including hemolytic anemia, thrombocytopenia, and schistocytes connected peripheral humor smears, which whitethorn mimic the symptoms of much terrible conditions; thrombotic microangiopathies, peculiarly thrombotic thrombocytopenic purpura (TTP) [4,5]. Differentiating betwixt the 2 conditions is captious since their absorption differs, ranging from parenteral vitamin B12 successful patients with PA with highly favorable outcomes to much precocious attraction with little favorable outcomes successful patients with TTP [3]. We contiguous the lawsuit of a 68-year-old antheral who initially presented with symptoms that mimicked TTP but were yet diagnosed arsenic having pseudo-TTP owed to vitamin B12 deficiency successful the discourse of PA. Following vitamin B12 treatment, helium developed reactive thrombocytosis.
Case Presentation
We contiguous a 68-year-old antheral with a past of intoxicant usage upset who presented to the exigency section (ED) with generalized weakness, fatigue, and shortness of breath. The diligent denied experiencing thorax pain, palpitations, dizziness, abdominal pain, nausea, vomiting, changes successful bowel habits, fever, and changes successful appetite. On presentation, the diligent had a humor unit of 80/50 mmHg, a pulse complaint of 95 beats per minute, a respiratory complaint of 16 cycles per minute, and a assemblage somesthesia of 98.6 F. The diligent was alert, awake, and oriented to time, place, and idiosyncratic during the examination. However, He appeared jaundiced, pale, weak, and stressed. There were nary evident murmurs successful S1 oregon S2 during the cardiac examination. During a thorax examination, determination are large vesicular sounds and close little lobe crackles. Abdominal introspection revealed nary hepatosplenomegaly oregon tenderness. A neurological introspection revealed nary abnormalities and nary swelling successful the little extremities.
ED investigations were important for terrible anemia, thrombocytopenia, mild leukocytosis, acute kidney injury, indirect hyperbilirubinemia, transaminitis, compensated metabolic acidosis with debased bicarbonate and elevated procalcitonin. Troponin, D-dimer, Lipase, and Ethanol levels were wrong mean range. Chest x-ray revealed close little lobe pneumonia. (Table 1).
Labs | value | Reference range |
CBC | ||
WBCs | 11.4 x103/MCL | 4.8-10.8 x103/MCL |
Hemoglobin (Hb) | 4 g/dL | 14-18 g/dL |
Hematocrit | 14.5% | 42-52 |
MCV | 120 fL | 80-99 |
Platelets (PLT) | 75x103/MCL | (150-450) x103/MCL |
KFT’s | ||
BUN | 30 mg/dL | 6-23 mg/dL |
Creatinine | 1.44 mg/dL | 0.5-1.2 mg/dL |
LFTs | ||
Total Bilirubin | 6.2 mg/dL | 0.0-1.2 mg/dL |
Direct Bilirubin | 1.8 mg/dL | 0.0-1.2 mg/dL |
ALT | 57 U/L | 0-33 U/L |
AST | 64 U/L | 5-35 U/L |
Coags | ||
INR | 1.6 | |
PT | 18.2 | 1-13 seconds |
ABG | ||
PH | 7:39 | 7.32-7.43 |
PCO2 | 30 | 38-41 mmHg |
HCO3 | 6 | 22-29 mEq/L |
procalcitonin level | 1.29 | < 0.25 ng/mL |
In the exigency department, the diligent received 2 liters of IV mean saline, Piperacillin-tazobactam 3.375 g IV, Vancomycin 1 gram IV, Paracetamol 975 mg tablet oral, Chlordiazepoxide 50 mg IV, and Pantoprazole 40 mg IV. The diligent received 1 packed RBC aft cross-matching was confirmed. The diligent was admitted to the intensive attraction portion (ICU) for attraction of pneumonia and terrible symptomatic anemia.
After infirmary admission, the diligent was transfused with 4 packed reddish humor cells and continued receiving IV antibiotics and IV fluids. Further humor enactment for anemia revealed debased serum iron, debased TIBC, precocious Ferritin level, debased serum vitamin B12 level, elevated reticulocyte percent, debased reticulocyte scale and mean implicit reticulocytic number (Table 2).
Lab | Value | Reference range |
Serum Iron | 28 mg/dL | 45-165 mg/dL |
TIBC | 134 mg/dL | 220-430 mg/dL |
Ferritin | 1,659 | 30 – 400 ng/mL |
Serum Vitamin B12 | 150 | 232-1245 pg/mL |
Reticulocyte % | 1.74 | 0.5-1.5 |
Rticulocyte index | 0.49 | 0.5-2.5 |
Absolute reticuloctic count | 0.0449 | 0.0221-0.0963 x 106 /MCL |
The abdominal US excluded biliary obstruction. Further investigations revealed LDH: 225 (n: 100-250 U/L), haptoglobin: 91 (n: 41-165) mg/dL, ADAMTS 13 enactment of 63% (n: 50%-100%) and schistocytes successful the peripheral humor smear. Anti-intrinsic origin antibody was importantly precocious astatine 218.6 AU/ML (n: 0.0-1.1), portion MMA and homocysteine were not collected. This diligent was diagnosed with PA based connected the operation of debased serum vitamin B12 and precocious anti-intrinsic origin antibody levels. Vitamin B12 was prescribed astatine a precocious dose of 1,000 ug iv regular successful the 5th time of admission. After stabilizing his condition, the diligent was transferred to the level for further management. The diligent was transferred from the ICU to a ward. Follow-up labs were explained successful Table 3.
CBC | ED | 1st day | 4th day | 9th day | 10th day | 11th day | 12th day | 13th day | 18th day | Reference |
WBCs | 11.4 | 10.87 | 12.05 | 14.26 | 12.87 | 10.22 | 10.22 | 11.69 | 9.29 | 4.8-10.8 |
Hb | 4 | 10.3 | 11.7 | 11.8 | 11.71 | 10.5 | 11.3 | 10.7 | 11.2 | 14-18 |
PLT | 75 | 329 | 831 | 12 | 1137 | 1075 | 936 | 911 | 509 | 150-450 |
In the discourse of PA with reactive thrombocytosis aft vitamin B12 correction, the diligent was diagnosed with pseudo-thrombotic microangiopathy owed to vitamin B12 deficiency Following the stabilization of the patient's condition, the diligent was discharged from the infirmary and instructed to travel up with the outpatient hematology clinic.
Discussion
TTP is characterized by acute hemolytic anemia, thrombocytopenia, bleeding factors, depletion, and organ damage. Vitamin B12 deficiency is associated with akin criteria [6]. Vitamin B12 plays a important relation successful the bony marrow's maturation of reddish humor cells. Decreased levels of B12 tin effect successful macrocytic hypochromic anemia, which leads to the demolition of peripheral humor RBCs. Vitamin B12 deficiency is associated with a wide scope of hematologic manifestations. Symptoms of vitamin B12 deficiency see anemia, leukopenia, thrombocytopenia, hyper-segmented neutrophils, and pancytopenia. The incidence of pseudo-TTP is estimated to beryllium 2.5% [7].
Andres et al. [8] studied the imaginable hematological complications of vitamin B12 deficiency successful 201 patients and described “pseudo” thrombotic microangiopathy successful 2.5 % of the studied populations. Tadakamalla et al. reported a lawsuit of a 31-year-old female with a past of fatigue and paresthesia successful some legs for 1 week. Indirect hyperbilirubinemia, thrombocytopenia, and schistocytes were recovered with subacute onset of fatigue and paresthesia, and TTP was suspected. However, platelet number did not amended with 4 days of plasmapheresis. Instead, aft the correction of debased vitamin B12, hematological complications improved, and much investigations revealed affirmative anti-intrinsic origin antibodies, which confirmed the diagnosis of PA associated pseudo-TTP [6].
A lawsuit bid person noted that pseudo-thrombotic microangiopathy is associated with prolonged PT, debased fibrinogen and platelets, schistocytes, and precocious LDH and D-dimer levels, arsenic good arsenic multi-organ dysfunction [9]. In 2017, Kandel et al. reported a lawsuit of PA presented with multi-organ dysfunction syndrome, diagnosed aboriginal arsenic pseudo-TTP [10].
The favoritism betwixt pseudo-TTP and TTP tin beryllium challenging. Both conditions whitethorn contiguous with hemolytic anemia, thrombocytopenia, and schistocytosis. By distinguishing betwixt the 2 conditions, patients with pseudo-TTP volition debar unnecessary interventions, and plasma transfusion complications tin beryllium reduced, arsenic pseudo-TTP does not respond to plasma exchange. [11].
Due to the therapeutic dilemma successful cases of TTP, which is simply a superior information that needs accelerated involution with plasma speech therapy [12], suggesting a acceptable of regular labs to beryllium done, including B12, methylmalonic acerb level, and reticulocyte count, to assistance separate TTP from Vitamin B12 associated pseudo-TTP.
It is indispensable to recognize that portion plasma speech tin temporarily relieve symptoms, vitamin B12 is the main enactment of treatment. In effect to vitamin B12 replacement, hemolysis markers typically alteration aft 1 to 2 days, and reticulocytes summation aft 3 to 4 days. In 85% of the cases, determination was a implicit effect to parenteral B12 replacement wrong 14 days, portion successful 15% of cases, it took up to six months for the diligent to execute a implicit response [13].
Reactive thrombocytosis for vitamin B12 tin happen. Ogston et al. described reactive thrombocytosis successful effect to treating thrombocytopenia and anemia. It should get backmost to mean wrong a tenable timeline [13]. Blood tests specified arsenic fibrinogen, hyperlipidemia, and D-dimer should besides beryllium evaluated to afloat measure the patient's viscosity situation. The plasma viscosity was not importantly antithetic betwixt groups contempt important differences successful platelet counts, according to Toprak et al. [14]. Due to the deficiency of a attraction requirement, consciousness of the anticipation of thrombocytosis pursuing vitamin B12 replenishment should beryllium raised. Close monitoring and reflection are the lone requirements.
Conclusions
Vitamin B12 deficiency pseudo-TTP is simply a improvement that should beryllium kept arsenic a differential diagnosis of therapy refractory TTP. Reactive thrombocytosis arsenic a effect to cobalamin therapy tin beryllium markedly precocious and needs further evaluation. Still, different inflammatory and bleeding markers are required for a implicit representation of the patient's homeostasis.
References
- Wathieu H, Bateman KM: A lawsuit of pseudothrombotic microangiopathy associated with pernicious anemia. J Gen Intern Med. 2021, 36:1775-7. 10.1007/s11606-020-06588-2
- Bizzaro N, Antico A: Diagnosis and classification of pernicious anemia. Autoimmun Rev. 2014, 13:565-8. 10.1016/j.autrev.2014.01.042
- Yousaf F, Spinowitz B, Charytan C, Galler M: Pernicious anemia associated cobalamin deficiency and thrombotic microangiopathy: lawsuit study and reappraisal of the literature. Case Rep Med. 2017, 2017:9410727. 10.1155/2017/9410727
- Stabler SP, Allen RH, Savage DG, Lindenbaum J: Clinical spectrum and diagnosis of cobalamin deficiency. Blood. 1990, 76:871-81. 10.1182/blood.V76.5.871.871
- Garderet L, Maury E, Lagrange M, Najman A, Offenstadt G, Guidet B: Schizocytosis successful pernicious anemia mimicking thrombotic thrombocytopenic purpura. Am J Med. 2003, 114:423-5. 10.1016/s0002-9343(03)00023-8
- Tadakamalla AK, Talluri SK, Besur S: Pseudo-thrombotic thrombocytopenic purpura: a uncommon presumption of pernicious anemia. N Am J Med Sci. 2011, 3:472-4. 10.4297/najms.2011.3472
- Panchabhai TS, Patil PD, Riley EC, Mitchell CK: When the representation is fragmented: vitamin B12 deficiency masquerading arsenic thrombotic thrombocytopenic purpura. Int J Crit Illn Inj Sci. 2016, 6:89-92. 10.4103/2229-5151.183026
- Andrès E, Affenberger S, Zimmer J, et al.: Current hematological findings successful cobalamin deficiency. A survey of 201 consecutive patients with documented cobalamin deficiency. Clin Lab Haematol. 2006, 28:50-6. 10.1111/j.1365-2257.2006.00755.x
- Walter K, Vaughn J, Martin D: Therapeutic dilemma successful the absorption of a diligent with the objective representation of TTP and terrible B12 deficiency. BMC Hematol. 2015, 15:16. 10.1186/s12878-015-0036-2
- Kandel S, Budhathoki N, Pandey S, et al.: Pseudo-thrombotic thrombocytopenic purpura presenting arsenic multi-organ dysfunction syndrome: a uncommon complication of pernicious anemia. SAGE Open Med Case Rep. 2017, 5:2050313X17713149. 10.1177/2050313X17713149
- Bauer PR, Ostermann M, Russell L, et al.: Plasma speech successful the intensive attraction unit: a communicative review. Intensive Care Med. 2022, 48:1382-96. 10.1007/s00134-022-06793-z
- Fahmawi Y, Campos Y, Khushman M, et al.: Vitamin B12 deficiency presenting arsenic pseudo-thrombotic microangiopathy: a lawsuit study and lit review. Clin Pharmacol. 2019, 11:127-31. 10.2147/CPAA.S207258
- Ogston D, Dawson AA: Thrombocytosis pursuing thrombocytopenia successful man. Postgrad Med J. 1969, 45:754-6. 10.1136/pgmj.45.530.754
- Toprak SK, Tek I, Karakuş S, Gök N, Kurşun N: Does reactive thrombocytosis observed successful robust deficiency anemia impact plasma viscosity?. Turk J Haematol. 2012, 29:248-53. 10.5505/tjh.2012.13008
Case report
peer-reviewed
Pernicious Anemia and Vitamin B12 Deficiency Presenting As Pseudothrombotic Microangiopathy and Developing Secondary Thrombocytopenia After Treatment: A Case Report
Author Information
Hazem Abosheaishaa
Internal Medicine, Icahn School of Medicine astatine Mount Sinai, Queens Hospital Center, New York City, USA
Internal Medicine/Gastroenterology, Cairo University, Cairo, EGY
Muhammad Ghallab
Internal Medicine, Icahn School of Medicine astatine Mount Sinai, NYC Health and Hospitals/Queens, New York City, USA
Mohammed Abdelwahed
Pathology, Donald and Barbara Zucker School of Medicine astatine Hofstra/Northwell, Uniondale, USA
Amr Ali
Pathology, Donald and Barbara Zucker School of Medicine astatine Hofstra/Northwell, Uniondale, USA
Eben Kimball
Internal Medicine, Queens Hospital Center, Jamaica, USA
Ethics Statement and Conflict of Interest Disclosures
Human subjects: Consent was obtained oregon waived by each participants successful this study. Conflicts of interest: In compliance with the ICMJE azygous disclosure form, each authors state the following: Payment/services info: All authors person declared that nary fiscal enactment was received from immoderate enactment for the submitted work. Financial relationships: All authors person declared that they person nary fiscal relationships astatine contiguous oregon wrong the erstwhile 3 years with immoderate organizations that mightiness person an involvement successful the submitted work. Other relationships: All authors person declared that determination are nary different relationships oregon activities that could look to person influenced the submitted work.
Article Information
DOI
10.7759/cureus.32095
Cite this nonfiction as:
Abosheaishaa H, Nassar M, Ghallab M, et al. (December 01, 2022) Pernicious Anemia and Vitamin B12 Deficiency Presenting As Pseudothrombotic Microangiopathy and Developing Secondary Thrombocytopenia After Treatment: A Case Report. Cureus 14(12): e32095. doi:10.7759/cureus.32095
Publication history
Peer reappraisal began: November 23, 2022
Peer reappraisal concluded: November 29, 2022
Published: December 01, 2022
Copyright
© Copyright 2022
Abosheaishaa et al. This is an unfastened entree nonfiction distributed nether the presumption of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction successful immoderate medium, provided the archetypal writer and root are credited.
License
This is an unfastened entree nonfiction distributed nether the presumption of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction successful immoderate medium, provided the archetypal writer and root are credited.
Case report
peer-reviewed
Pernicious Anemia and Vitamin B12 Deficiency Presenting As Pseudothrombotic Microangiopathy and Developing Secondary Thrombocytopenia After Treatment: A Case Report
Figures etc.
Labs | value | Reference range |
CBC | ||
WBCs | 11.4 x103/MCL | 4.8-10.8 x103/MCL |
Hemoglobin (Hb) | 4 g/dL | 14-18 g/dL |
Hematocrit | 14.5% | 42-52 |
MCV | 120 fL | 80-99 |
Platelets (PLT) | 75x103/MCL | (150-450) x103/MCL |
KFT’s | ||
BUN | 30 mg/dL | 6-23 mg/dL |
Creatinine | 1.44 mg/dL | 0.5-1.2 mg/dL |
LFTs | ||
Total Bilirubin | 6.2 mg/dL | 0.0-1.2 mg/dL |
Direct Bilirubin | 1.8 mg/dL | 0.0-1.2 mg/dL |
ALT | 57 U/L | 0-33 U/L |
AST | 64 U/L | 5-35 U/L |
Coags | ||
INR | 1.6 | |
PT | 18.2 | 1-13 seconds |
ABG | ||
PH | 7:39 | 7.32-7.43 |
PCO2 | 30 | 38-41 mmHg |
HCO3 | 6 | 22-29 mEq/L |
procalcitonin level | 1.29 | < 0.25 ng/mL |
Lab | Value | Reference range |
Serum Iron | 28 mg/dL | 45-165 mg/dL |
TIBC | 134 mg/dL | 220-430 mg/dL |
Ferritin | 1,659 | 30 – 400 ng/mL |
Serum Vitamin B12 | 150 | 232-1245 pg/mL |
Reticulocyte % | 1.74 | 0.5-1.5 |
Rticulocyte index | 0.49 | 0.5-2.5 |
Absolute reticuloctic count | 0.0449 | 0.0221-0.0963 x 106 /MCL |
CBC | ED | 1st day | 4th day | 9th day | 10th day | 11th day | 12th day | 13th day | 18th day | Reference |
WBCs | 11.4 | 10.87 | 12.05 | 14.26 | 12.87 | 10.22 | 10.22 | 11.69 | 9.29 | 4.8-10.8 |
Hb | 4 | 10.3 | 11.7 | 11.8 | 11.71 | 10.5 | 11.3 | 10.7 | 11.2 | 14-18 |
PLT | 75 | 329 | 831 | 12 | 1137 | 1075 | 936 | 911 | 509 | 150-450 |