Challenging and perplexing case of hemolytic anemia today! Two entities that were discussed are highlighted below:
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Rare! 1-10 cases per per million people
- Caused by an abnormality of the GPI anchoring protein that protects the RBCs from complement
- Test for CD55 and CD59 with flow cytometry
- Classic presentation is unexplained hemolytic anemia, but can be associated with aplastic anemia and can have increased risk of thrombosis (hypercoagulable due to free Hgb in blood or other mechanisms?).
- This is usually an acquired condition
- Turns out that hemolysis can occur throughout the day and not just at night. Often there can be triggers causing hemolysis.
- Think about PNH when you have a patient with a hemolytic anemia that is DAT negative
Paroxysmal Cold Hemoglobinuria (PCH)
- Caused by an IgG Ab that fixes complement in the cold, but only causes intravascular hemolysis upon rewarming
- Classic presentation is hemoglobinuria occurring after patient exposure to cold
- DAT should be positive for complement and negative for IgG
- There is also a separate but similar entity of autoimmune hemolytic anemia due to IgM cold agglutinins. This is often triggered by infection or underlying lymphoproliferative disorder and can also cause intravascular hemolysis. Watch out for hyperviscosity with IgM!
Evernote link: http://www.evernote.com/l/APi1T7y8ENhA84y2C28Z-y8n7zxBuHdRSdg/
Next time you are in the ICU have someone show you the sub-xyphoid view with the bedside ultrasound which can be a great way for assessing for an effusion. A nice demonstration of the view from YouTube is here and includes both normal and abnormal including what it looks like when there is an effusion!!!
Remember to keep in mind the complications of endomyocardial biopsy which include:
– myocardial perforation with pericardial tamponade
– heart block
– arterial puncture
– pulmonary embolization
– nerve block or injury
– damage to the tricuspid valve
– creation of arteriovenous fistula
– deep venous thrombosis
Also, don’t forget that most patients with cardiac tamponade will have elevated jugular venous pressure but if you don’t look for it you won’t see it!!
What is it?
- Inflammation of the subcutaneous fat
- The form of panniculitis most people have heard of is Erythema nodosum, which itself has a broad differential (up to 55% of cases are idiopathic, and don’t forget to think about drugs!)
Give me a framework to think of panniculitis!
- There is a histologic framework of thinking of panniculitis, based on the underlying architecture of fat. It can either be “septal” panniculitis (fibrous septa between fat lobules) or “lobular” panniculitis (fat lobules themselves)
- A more helpful framework for us non-histologically inclined IM residents is the following (courtesy of UpToDate and this review from Harry: PMID 25268197)
- Inflammatory: erythema nodosum, sclerosing panniculitis, lupus panniculitis, cutaneous polyarteritis nodosa, erythema induratum (non-TB versions)
- Infection: can be caused by many microorganisms. Can be direct inoculation (primary) or hematogenous spread (secondary).
- Bacterial (Strep, Nocardia, Brucella, Pseudomonas, Staph, Klebsiella)
- Mycobacterial (TB, NTB)
- Fungal (Candida, Aspergillus, Fusarium, Histo)
- Deposition: calciphylaxis, gout (rare)
- Enzymatic destruction: pancreatic panniculitis (a.k.a. pancreatic fat necrosis), alpha-1 antitrypsin deficiency panniculitis
- Malignancy: subcutaneous lymphoma
Evernote link: http://www.evernote.com/l/APgkLpOp775IqrwDOb_SumS1xHYqP-rIvhw/
Take home pearls from the case
- Lobar nephronia is a radiologic term for acute focal bacterial nephritis. The term refers to a renal mass caused by acute focal infection, as in pyelonephritis, that occurs without the liquefaction that usually leads to full abscess formation.
- While surgical intervention is not always required, drainage of the lobar nephronia should be considered if the patient does not respond promptly to appropriate antibiotics.
- Imaging in pyelonephritis is recommended for the following patients:
- Persistent symptoms after 48-72 hours of antibiotics
- Patients with renal colic symptoms or history of stones
- Prior urologic surgery
- Repeated episodes of pyelo/urosepsis
- For your young women presenting with flank pain, keep Loin pain hematuria syndrome on your differential! LPHS is a constellation of recurrent episodes of several flank pain accompanied by gross or microscopic hematuria in the absence of other identifiable causes. The exact pathophys is unknown, but likely involves glomerular hemorrhage. (Consider using the term “loin” instead of flank in your future rounding presentations just to see how people react, except human loins are actually that side area below the ribs and along your hips).
Dube GK, Hamilton SE, Ratner LE, et al. Loin pain hematuria syndrome. Kidney Int 2006; 70:2152.
Rosenfield AT, Glickman MG, Taylor KJ, Crade M, Hodson J. Acute focal bacterial nephritis (acute lobar nephronia). Radiology. 1979 Sep; 123(3):553-61.
Kanel KT, Kroboth FJ, Schwentker FN, Lecky JW. The intravenous pyelogram in acute pyelonephritis. Arch Intern Med 1988; 148:2144.
Evernote link here: https://www.evernote.com/shard/s300/sh/b01a1b3c-1e0d-43d8-8129-ff9c662e0b68/e4d203c67d4248f816cb2b024d9807aa
Think of all the usual causes of AKI in a non-transplanted patient PLUS the following additional list:
Mnemonic from Dr. Allison Webber (UCSF transplant nephrology) of Serum CReatinine Increasing.
- Structural (immediate post-transplant period)
- Vascular: renal artery stenosis, venous thrombus, anastamosis issue
- Obstructive: urinoma, hematoma, lymphocele
- Ureteral stricture can present later (distal ureter can gradually become ischemic and stricture)
- Calcineurin inhibitor toxicity
- Antibody mediated
- Cell mediated
- Recurrence of primary disease (e.g. FSGS)
- Infection (remember that infections such as pyelo in a normal patient don’t cause AKI, but it can in a post-transplant patient with a single functional kidney)
- regular infections
- opportunistic infections in immunocompromised host
Dysphagia – a simple approach
The first step in evaluating dysphagia is to determine whether the patient has oropharyngeal dysphagia or esophageal dysphagia. The diagnostic algorithm branches here!
Oropharyngeal dysphagia (aka “transfer dysphagia”): characterized by difficulty initiating a swallow
Esophageal dysphagia: characterized by trouble swallowing several seconds after the swallow has been initiated, and a sensation of food getting stuck in the esophagus.
Patients with esophageal dysphagia should be referred for an upper endoscopy!
Who should get a barium swallow study beforehand?
Patients at increased risk of perforation during upper endoscopy should have a barium swallow study performed before endoscopy. Risk factors include:
– prior surgery for pharyngeal/laryngeal cancer
– a known complex stricture
– history of radiation or caustic injury to the esophagus
Risk Factors for Esophageal Candidiasis:
– HIV (most common risk factor by far)
– Less well-studied/established risk factors include:
– idiopathic CD4 lymphopenia
– hematologic malignancies
– systemic corticosteroids
– inhaled corticosteroids
– increasing age
Last quick pearl: The hallmark of esophageal candidiasis is odynophagia! Patients are usually able to localize the pain to a discrete retrosternal area.
Take home pearls from the case:
- Stage IV disease is defined as having one of the following: malignant pleural effusion, parenchymal metastases (not just on surface of intestines, omentum, etc) or mets to extra-abdominal organs or lymph nodes
o Ovarian cancer treatment updates hot off the presses – after cytoreductive surgery, intraperitoneal chemo with IV chemo for stage III ovarian cancer can improve survival significantly, but <50% of patients actually receive this! http://jco.ascopubs.org/content/early/2015/08/03/JCO.2015.61.4776
- The underlying malignancies associated with dermatomyositis vary by prevalence of certain cancers in specific populations (for example, nasopharyngeal carcinoma in highly associated in Southeast Asian patients, but not in other parts of the world). However, adenocarcinomas of the lung, cervix, ovaries, pancreas and bladder account for over 2/3 of the associated cancers, and some studies suggest ovarian cancer has an even stronger association.
- Ovarian cancer is associated with a wide variety of paraneoplastic syndromes involving nearly all organ systems, and DIC is among these associated syndromes.
- Solid tumors generally have a very low risk of tumor lysis syndrome, and this is even less likely to develop after the patient has undergone cycles of chemotherapy
Shanbhogue AK, Shanbhogue DK, Prasad SR, et al. Clinical syndromes associated with ovarian neoplasms: a comprehensive review. Radiographics 2010; 30:903.
Sigurgeirsson B, Lindelöf B, Edhag O, Allander E. Risk of cancer in patients with dermatomyositis or polymyositis. A population-based study. N Engl J Med 1992; 326:363.
Cairo MS, Coiffier B, Reiter A, et al. Recommendations for the evaluation of risk and prophylaxis of tumour lysis syndrome (TLS) in adults and children with malignant diseases: an expert TLS panel consensus. Br J Haematol 2010; 149:578.