Category Archives: #Heme/Onc

Friday MR with Justin: Scurvy!

A middle aged woman w/ PMHx Bipolar, HLD, and hypothyroidism who p/w syncope in s/o 2-3 months of LE ecchymoses/petechiae, admitted for syncope, anemia, and petechial rash ultimately diagnosed with scurvy.

 

Key Learning Points

-Vitamin C deficiency results in impaired collagen synthesis – typical pathologic manifestations occur in collagen-containing tissues/organs: skin, cartilage, dentin, osteoid, and capillary blood vessels – scurvy may present with gum bleeding, petechiae or ecchymoses

-The skin findings may initially resemble a vasculitis!

-Treatment is vitamin C supplementation – many of the constitutional symptoms improve within 24 hours – bruising and gingival bleeding resolve within a few weeks

MR with Shawn Tate + Kleckner: Large Granular Lymphocyte Leukemia

A middle aged man with hx of DM2, BPH, multiple recent infections, who presented with Neutropenic fever with ANC of 0 and cellulitis of Left foot.  After multiple hospitalizations was diagnosed with Large Granular Lymphocyte Leukemia (LGL Leukemia).

Teaching points:
– Heme/Onc cases in general are often difficult due to our exposure and the rarity of cares.
– LGL Leukemia is rare, estimated to be 1 in 10 million in US
– Must differentiate this condition from reactive LGL expansions and chronic lymphomatous conditions, as well as chronic and aggressive NK cell leukemias.
– It is thought that autoimmunity plays a central role in its development
Associated with infections (EBV, CMV) as well as RA, Schogren’s and other autoimmune conditions.  40% of cases are associated with RA or an autoimmune cytopenia (pure red cell aplasia, autoimmune hemolytic anemia, ITP.
– Diagnosis is clinical with undefined neutropenia, confirmed with bone marrow biopsy with increased number of LGLs (normal 200-400 micro/L).
– Treatment is primarily immune suppression with MTX, cyclophosphamide, Cyclosporine, +/- steroids. (this patient also received Neupogen with good response)
– Good prognosis, 85% survival at 4 years

Friday live case MR with Justin Louie: Hemolysis

A middle aged woman with history of bipolar disorder (on lamotrigine, escitalopram, buproprion, and olanzapine)presenting after a syncopal episode in the setting of 2.5 months of petechiae, macules, and ecchymosis, found to have an acute normocytic anemia. Work up revealed an elevated LDH, D-dimer, and reticulocyte count. Autoimmune labs were unremarkable. Skin biopsy is pending.

Kinetic approach to anemia

->Decrease in RBC production (low retic)

->Blood loss

->Increased destruction of RBCs

o   Intravascular hemolysis  (MAHA, PNH, clostridium perfringens, cold agglutinin disease, paroxysmal cold hemoglobinuria)

– Often increased LDH, indirect bili, and decreased hapto

– Clinical observations: jaundice, renal damage, dark urine (hgb), acrocyanosis, livedo reticularis

– Additional lab studies: free hemoglobin in plasma (hemoglobinemia), hemoglobinuria, hemosiderin in stained urine sediment (if ongoing for at least a week)

Algorithm take aways: 1) When to consider urgent treatment 2) Transfusion related? 3) Get a direct-coombs 4) schistocytes suggest hemolysis, but the sensitivity is not 100%

9/13 Tuesday case presentation with JLo: Hypereosinophilic syndrome

An elderly man who initially presented with 10 days of loose stools and abdominal cramping and no red flags symptoms.  He represented a week later with weight loss, diffuse itching and rash.  A CBC was significant for eosinophilia, and through further workup he was diagnosed with hypereosinophilic syndrome

Learning Points

-DDx of diarrhea -> secretory/osmotic, inflammatory, malabsorptive

-Practical tip on differentiating osmotic vs. secretory causes of diarrhea: osmotic diarrhea will cease with fasting

-Hypereosinophilic syndromes (HES) are disorders marked by the sustained overproduction of eosinophils, associated with damage to one or more organs due to eosinophilic infiltration and mediator release

-Hypereosinophilic conditions can be restricted to one organ and overlap of hypereosinophilic conditions can exist – eosinophilic GI disorders, chronic eosinophilic pneumonia, and Well’s syndrome [dermatologic involvement]

-The following organs can be involved: dermatologic (37%), pulmonary (25%), gastrointestinal (14%), cardiac (5%), and neurologic (4%)

-Oncologic workup for HES includes bone marrow aspiration/biopsy – looking for a > 20 % of eosinophils in addition to specialized studies to ID patients with myeloproliferative or clonal lymphocytic variants

-HES is treated with steroids

8/29 MR with Brad Lewis: Severe thrombocytopenia

An elderly male who works as a car mechanic presents with 3 weeks of rash and 1 week of epistaxis and mucosal bleeding, found to have thrombocytopenia with plt 1 and anemia with hemoglobin of 7.4. He was initially treated for ITP (with the thought that his anemia was just 2/2 bleeding), but due to his persistent cytopenias and inappropriately low reticulocyte count, suspicion was high for a bone marrow process; he ultimately was diagnosed with AML.
Take-home points for thrombocytopenia from Brad Lewis:
1) Schistocytes on a smear are only helpful if you see them. If you don’t see them, you still can’t definitively rule out your MAHAs (DIC, TTP, etc) so interpret along with other labs (LDH, haptoglobin, bili) and the overall clinical picture
2) Pay attention to the rest of the smear, and ask heme to help interpret – even a rare myelocyte or blast or nucleated RBC is significant.
3) Get that reticulocyte count, AND trend it! Often the trend will help more than a single value.
 
Help! I never know how to interpret a reticulocyte count.
Remember this rough graph to help you estimate if a reticulocyte count is inappropriately low (i.e. not high enough given the degree of anemia)

7/29 Morning Report w/ Moyukh + Dr. Lopez: Atypical HUS

A middle aged man found down presenting w/ AMS, ARF, and microangiopathic hemolytic anemia. He was initially presumed to have TTP and emergently received plasmapheresis; several days later, the ADAMSTS13 test actually returned normal, and the diagnosis of atypical HUS was made. He was ultimately discharged on eculizumab.
*Take away points 1) TTP is the most life-threatening, don’t miss dx among the MAHAs;  2) Very high creatinine elevations above 5 or so are more consistent with HUS than TTP, however most of these patients will receive plasmapheresis until diagnostic tests can rule out TTP; 3) Atypical HUS is a complement-mediated MAHA, and treatment with eculizumab impairs the complement cascade, hence when starting treatment these patients are at a VERY high risk for meningococcal disease and should be vaccinated + receive abx prophylaxis*
Diagnostic algorithm in differentiating TTP and HUS:
1) If initial presentation is consistent with TTP (MAHA including elevated LDH, schistos + renal failure, AMS) –> emergent plasmapheresis
2) If ADAMSTS13 returns normal, dx is likely HUS –> stop plasmapheresis + supportive care
3) If no history of preceding GI illness, dx is likely atypical HUS –> eculizumab