Clinical Presentation

Clinical Presentation

By JoAnne Bang, PGY 3

History and Physical

55 year old female patient with PMHx of CHF, allergic asthma and HLD presents to the ED with worsening of shortness of breath. Patient reports that she was in the hospital few weeks ago, was recommended Left and Heart cath, but pt declined because she wanted to come see her regular Cardiologist. Hence, patient was discharged with recommendation to follow up with Cardiology in 2- 3 days. However, patient did not follow up and comes in to the ED for worsening of SOB and with complaint of dark urine, diffuse rash that appeared in past 2- 3 weeks. .

Vitals and Physical exam

Constitutional: appears fatigued, unable to lay flat due to shortness of breath. CV: tachycardia without irregular or skipped beats, murmur . Lung: decreased breath sound Abd: unremarkable abdominal exam. Skin: diffuse petechial rash in bilateral UE, LE and trunk. Neuro: alert, oriented x 2, normal sensation and symmetrical muscle strength. MSK: Bilateral pitting edema

Past Medical History

• Hypertension

• Congestive Heart failure
• Allergic asthma
• Hyperlipidemia

• Smoker - smoked a pack/day for 25 years, but cut back tro 1-2 pack per week for about 15 years ago
• Heavy use of alcohol for about 15 years

Past Social History

• Mother - Passed away from Lung cancer
• Father - healthy
• Brother - HF

Past Family History

What is Differential diagnosis for this patient?

• Shortness of breath
• Acute on chronic heart failure
• Asthma exacerbation
• Pneumonia
• Pulmonary embolism
• Dark urine with Petechial rash
• Hemolytic uremic syndrome
• Thrombotic thromocytopenic purpura
• Autoimmune hemolytic anemia
• Acute viral hepatitis ...etc

Differential diagnosis

What Labs and imaging would you order?

Petechial rash work up

• CBC with peripheral smear
• Urinalysis
• Coagulation studies - haptoglobin, reticulocyte counte, copper S,
• Protime/INR, Fibrinogen
• Renal function tests
• Liver function panel
• TSH
• LDH
• B12/Folate

Labs and Imaging

• Chest Xray
• CT PE protocol

Shortness of breath imaging

Shortness of breath work up

• BMP
• BNP
• D-dimer

Labs and Imaging

• Haptoglobin at 43
• Reticulocyte 2.6, Absolute reticulofcyte 124.30
• Acute hepatitis panel - all non reactive
• Copper S 267 (elevated)
• LDH elevated 557

• Direct antiglobulin test (IgG and C3)
• DAT IgG positive
• DAT C3, negative
• Peripheral smear: mild abosulte neutrophilia, monocytosis. Thrombocytopenia. Erythrocytes are microcytic.

• CBC with diff
• everything wnl except:
• MCV 79.2, MCH 24.7 RDW 22.6
• Platelet count 99 (one month ago, 238)
• Neutrophil 7.7

Results

CT PE protocol - positive for PE in RLL pulmonary artery brasnches and pulmonary inmfarct in the medial basilar segment of right lower lobe. Also, noted tro have patchy nodule areas of consolidation in lower lung fields that could have been indicative of pneumonia Chest Xray - showed consolidation (as similar to CT PE protocol)

Results

Patient was admitted for treatment and work up for: - Acute on chronic CHF - Pulmonary embolism - Concern for pneumonia - Thrombocytopenia - Diffuse petechial rash - Altered mental status (encephalopathy)

Dispo?

Acute on chronic CHF - Cardiology was consulted. - Pt was started on IV diurectic - IV Lasix 80 mg twice daily and added Aldactone. - TEE was done, showed EF 40-45% with severe aortic stenosis. Unprovoked Pulmonary embolism - Patient was started on IV heparin - Started/continued on O2 supplementation Pneumonia - Started on IV Unasyn per ID recommendation. Petechial rash with thrombocytopenia - Heme/Onc and Infectiouse disease consulted. - Given + Coomb test, pateint was started on 1 mg/kg/day of Prednisone Altered mental status - Continues, does not improve much

Hospital course

Diffuse Petechial Sore/Rash - ID consulted. - Concern for VZV or HSV - Started on Ig of Valtrex 3 times daily to cover possible viral etiology for diffuse sores. - General surgery was consulted for biopsy of the lesion.

Hospital course

Day 7 at hospital

Acute on chronic CHF - Patient appeared to be minimally improve after IV diuretic. - BNP continues to rise at 9539 from 8117 (at admission) - Echo repeated. - Echo showed EF of 25% with severly redued systolic function, severe hypokinesis various parts of walls of the heart, aortic valve - severely calcified, severe aortic stenosis Pulmonary embolism - Continued on IV heparin given possible plan for Left and right heart cath due to severe aortic stenosis. - Continued on Oxygen Petechial rash with thrombocytopenia - Thrombocytopenia - trending upward - Petechial rash continues to remain but improve - Heme/onc recommoned continued work up with ID given heme/onc believed rash to be related to viral/vasculitis rash. - Biopsy result - no obvious pathology - Per ID, started Doxycycline 100 mg BID, Lyme titer, Ehrlichia, Anaplasma

Hospital course

After discussion with family, decided to transfer to Cleveland Clinic due to decompensated HF and complex care management.

Hospital course

Decompensated HF - Continued on Lasix 80 IV BID and Sprinolactone 25 mg daily - Continued on metoprolol 25 BID Aortic insufficiency with concern for ischemic cardiomyopathy given global hypokinesis on repeat echo - Repeat TEE was ordered. - Plan for LHC vs. CT coronary angiogprahyh Pulmonary embolism - Continued on IV heparin given possible plan for Left and right heart cath due to severe aortic stenosis. - Continued on Oxygen Acute respiratory insufficency - CXR and BiPAP ordered. - Cefepime/vanc was started for concern for PNA on chest xray

At Cleveland

Petechial rash - Derm and ID consulted -Repeat biopsy ordered - C3, C4, and UA ordered for vasculitis work up - VZV/HSV swab ordered. - Vitamin C (scurvy) level ordered - Blood cultures ordered. AIHA/Thrombocytopenia - Stopped Prenisone - Ordered repeat hemolysis work up - ID consulted

At Cleveland

Acute Immune Hemolytic Anemia

A type of anemia that occurs when the immune syshtem mistakenly destroys red blood cells at a rate faster than they can be replaced.

Epidemiology of AIHA

Incidence: Estimated to be 1-3 cases per 100,000 people per year. Age/Gender distribution:

• Warm AIHA - most common in adults age 40-70 years old, more common in female.
• Cold AIHA - more common in older adults, > 60 years of age, more common in male

Mortality: prognosis can be good with appropriate treatment. However, relapse can occur and if complicated bly other conditions, mortality can be higher.

Alloimmune hemolytic anemia

blood transfusion

Drug Induced AIHA

- Medications such as penicillin, cephalosporins, methydopa can sometimes cause this reaction

- Infections (ex. Mycoplasma, EBV) - Lymphoproliferative disorders

Cold AIHA

Etiology of AIHA

Warm AIHA

- Autoimmune disease - Lymphoproliferative disorders - Idiopathic - Malingnancy

• Also, may present with signs of underlying disease
• Such as malar rash, joint deformities, or oral ulcers (signs of Lupus)

Signs and symptoms

• Fatigue
• Pallor
• Jaundice
• Dark urine
• Shortness of breath
• Tachycardia
• Splenomegaly or Hepatomegaly

Diagnosis and Work up

• Complete Blood Count (CBC): Shows anemia with a low hemoglobin level and elevated reticulocyte count (indicating that the body is trying to produce more red blood cells).
• Peripheral Blood Smear: May show fragmented red blood cells (schistocytes) or spherocytes (in the case of WAIHA).
• Direct Antiglobulin Test (DAT), also called Coombs test: This is a key test that detects antibodies or complement proteins on the surface of red blood cells, confirming the diagnosis of AIHA.
• Haptoglobin levels: Decreased haptoglobin levels, which are proteins that bind free hemoglobin, are often seen in hemolytic anemias.
• Reticulocyte count: An elevated reticulocyte count suggests that the bone marrow is responding to the increased destruction of red blood cells. (Differentiating factor from other conditions)

Treatment

• The treatment of AIHA depends on the severity and underlying cause. Common approaches include:
• Corticosteroids
• Immunosuppressive drugs: If corticosteroids are not effective, drugs like azathioprine, cyclophosphamide, or rituximab may be used.
• Blood transfusions: In cases of severe anemia, blood transfusions may be necessary to provide the body with adequate red blood cells.
• Splenectomy (removal of the spleen): In some cases, if the spleen is the primary site of red blood cell destruction, removing it may improve the condition.
• Treatment of underlying conditions: If AIHA is secondary to an underlying condition (e.g., infection or malignancy), treating the underlying disease can help manage the anemia.

Thank you!