Heme Onc [MICROCYTIC ANEMIA]

Brief Introduction
So we know the patient is anemic; we saw the MCV was low. If CBC
they were unstable we’d transfuse them. But we ought to get
some labs first because after transfusion the labs will be based on MCV
the transfused blood only. Step one is to get Iron Studies and go
from there.
Microcytosis
Iron Deficiency Anemia
Iron Deficiency Anemia
The most common form of microcytic anemia is iron deficiency.
The normal requirement of iron is 1mg/day with a maximum of Fe Studies Anemia of Chronic Disease
3mg/day. If the body starts to lose blood it may begin using iron Thalassemias
(to replace the lost hemoglobin) at a greater rate than it can be
absorbed. But this also means that it must be a chronic source of
Iron Stores Sideroblastic Anemia
blood loss. Potential causes are GI Bleeds (slow, polyps,
↑TIBC
hemorrhoids, etc) or Gynecologic losses (menorrhagia, cancer).
(Available Storage)
Alternatively, decreased uptake of iron in a non-bleeding person
(as in a gastrectomy) is possible. In any male or postmenopausal ↓Fe
female with iron deficiency anemia follow up with a colonoscopy (Iron in the Blood)
to rule out cancer. The best test to diagnose iron deficiency
↓Ferritin
anemia is a Bone Marrow Biopsy. But it’s rarely done because
(Iron in the Stores)
Iron studies are so good at diagnosing Iron Deficiency Anemia.
The most sensitive part of the Iron studies is a low Ferritin (if Iron Deficiency Anemia. Iron stores are depleted, plenty of
Ferritin is low, it’s iron deficiency anemia, period). That is, the storage availability. Iron is low. ↑TIBC, ↓Ferritin, ↓Fe.
iron stores are small. Low stores means high capacity to bind, so
there’ll be an elevated TIBC. The low stores also means low
serum iron. Stop the bleeding then give iron. It takes 6 weeks to
replace the serum iron and 6 months to replace iron stores.

Anemia of Chronic Disease

When there’s inflammation the body is trying to prevent
Iron Stores
whatever it’s fighting from getting the iron it needs. If it’s only an
acute process, that helps fight infection. A side effect is that it ↓TIBC
makes the iron unavailable even to the host! Great in fighting an (Available Storage)
infection; awful in a chronic disease. Essentially, what happens ↓Fe
is the connection between the Iron stores and the blood is severed. (Iron in the Blood)
The body has a lot of iron stored so a low capacity to bind but
still has a low serum iron. Treating the underlying disease will ↑Ferritin
fix the anemia (the inflammation goes away, the iron stores can (Iron in the Stores)
be reconnected to the blood). Sometimes, that’s not possible
(Lupus, Rheumatoid Arthritis) so help the body utilize iron stores Anemia of Chronic Disease. There’s a disconnect between
with EPO. the blood and the iron stores, but iron absorption is intact.
↓TIBC, ↑Ferritin, ↓Fe
Thalassemia
Something different is going on in thalassemia. It’s not the iron
stores that are the problem - it’s the hemoglobin. There’s a
genetic disease (α, chromosome 16, frameshift and β,
chromosome 11, deletion) that leads to ↓ production of the normal β-Thal α-Thal
hemoglobin with 2α and 2β; HgbA1 α2β2. It doesn’t matter which HgbA1 α2β2
Asx N/A 1 Gene Deleted
portion is broken - the patient is going to have anemia with Minor 1 Gene Deleted 2 Gene Deleted HgbA2 α2δ2
normal iron studies. The way to definitively diagnose Major 2 Gene Deleted 3 Gene Deleted HgbF α2у 2
thalassemia is with a Dead N/A 4 Gene Deleted Barts y4
HgbH β4

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Heme Onc [MICROCYTIC ANEMIA]

Hemoglobin Electrophoresis (α-thal is ‘normal’). Here’s the Iron Stores
TIBC Normal
kicker; because anemia is based on severity, not etiology,
(Available Storage)
definitive diagnosis is not required except for genetic counseling.
Think of ALL thalassemia patients as minor (do nothing) and Fe Normal
major (routine transfusion). The deal with which hemoglobin it (Iron in the Blood)
is, 1, 2, 3, 4 gene deleted is unnecessary and bogus for the clinical
rotations. Recognize the hemoglobins (A1, A2, Fetal, Barts, Ferritin Normal
HbH) but realize it’s either do nothing (minor) vs transfuse (Iron in the Stores)
(major). Each bag of blood has 350mg Fe - enough supply for one
year. Frequent transfusion leads to iron overload treated with Thalassemia. The iron stories are normal. The more genes
deferoxamine to prevent Hemosiderosis. Deferasirox is an oral deleted, the more severe the disease. Consider
medication that might pop up on a test or on the wards. Thalassemias as either minor or major only.

Sideroblastic Anemia
Nobody likes Sideroblastic anemia because it’s “hard.” Really Iron Stores
it’s because it sounds terrifying and is named from what it looks Normal TIBC
like on Bone Marrow Biopsy. It’s the only microcytic anemia (Available Storage)
with elevated iron. Definitively diagnose it with a bone
marrow biopsy, which will show the ringed sideroblasts. It ↑Fe
has a number of causes (Lead, EtOH, Isoniazid, a pyridoxine (Iron in the Blood)
metabolic disease of B6, and Myelodysplasia / AML). Get the
pt away from lead, give them B6, and do a BM Bx for the
cancer (which, coincidentally, you just did for the diagnosis). Normal Ferritin
(Iron in the Stores)
Sideroblastic. Diagnosis of Exclusion confirmed on bone
marrow biopsy. The tipoff is an elevated iron despite an
anemia with small cells

Anemia Pathology Ferritin TIBC Iron Best Test Tx f/u

Iron Deficiency Blood Loss (Chronic) ↓Ferritin ↑TIBC ↓ Fe BM Bx Iron Colonoscopy
GI, GYN
Anemia of Any chronic ↑Ferritin ↓TIBC ↓ Fe BM Bx Treat the Dz (Steroids) -
Chronic inflammatory disease Try Epo
Disease
Thalassemia Chr 16, α, Frameshift Normal Normal Normal Hgb Minor: Ø Deferoxamine
Chr 11, β, Deletion Ferritin TIBC Iron Electrophoresis Major: Transfuse (transfusions)
Sideroblastic Lead, B6, genetic Dz, Normal Normal ↑ Fe BM Bx Give B6, Look for -
Myelodysplasia, Ferritin TIBC (Ringed Cancer
EtOH, ↓ Copper Sideroblasts)

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